True polycythemia: causes, symptoms, diagnosis, treatment. Classification, symptoms and treatment of polycythemia What is polycythemia

All symptoms of true polycythemia are combined into two syndrome (a stable set of symptoms united by a single development).

• Pletic syndrome (Full-blooded - related to the enhancement of the blood circulation):
  • headaches;
  • dizziness;
  • grace, compressing sore of the sternum during exercise;
  • heaviness in the head;
  • erythrocianoz (redness of the skin with a shiny tint);
  • "Rabbit eyes" (redness of the eye arising from the expansion of their vessels);
  • the severity in the left top of the abdomen due to an increase in the spleen;
  • skin itching, amplifying after water procedures (arises due to irritation of nervous endings of the skin by the spree of red blood cells - red blood cells);
  • an increase in blood pressure, slightly downwards with the help of standard treatment, but reduced by means of bleeding (removal from the body of a certain amount of blood with therapeutic purposes);
  • erythrolealgia is sharp burning pains in the tips of the fingers, decreasing after the reception of drugs that dilute blood. It can manifest as a kind of swelling with redness of the lower third of the leg or foot, accompanied by a sharp pain.

• Myeloproliferative syndrome (associated with the increased formation of erythrocytes (red blood cells), platelets (blood plates) and leukocytes (white blood cells)):
  • bone pain;
  • the severity in the upper part of the belly to the left by increasing the spleen;
  • the severity in the top of the belly to the right due to the increase in the liver;
  • general weakness;
  • fatigue;
  • increase body temperature.

Forms

Several stages of true polycythemia are distinguished:

• I - initial stage.
  • Lasts 5 years or more.
  • Moderate manifestations of the pilor syndrome (that is, the full-blooded, associated with the enhancement of blood circulating organs).
  • The spleen is not enlarged.
  • In general blood test, a moderate increase in the number of erythrocytes (red blood cells) is observed, in the bone marrow - enhancing the formation of erythrocytes or all blood cells, except for lymphocytes (a special version of leukocytes - white blood cells).
  • Complications rarely occur.
  
  
• II A - polycytemic (that is, with an increase in the number of all blood cells) stage.
  • Lasts 5-15 years.
  • A pronounced plaque syndrome, an increase in the liver and spleen, thrombosis (closing of the lumen of the vessel with blood cloth - blood clot) of the arteries and veins, bleeding.
  • An increase in the spleen is due to its increased blood flow and the destruction of an increased amount of blood cells.
  • There are no sections of tumor growth in the spleen.
  • Bleeding can lead to iron deficiency (metal used for the formation of hemoglobin - a special substance of erythrocytes carrying oxygen).
  • In general blood test, the increase in the number of erythrocytes, platelets (blood plates), leukocytes (except lymphocytes) is detected.
  • In the bone marrow - strengthening the formation of all blood cells, except lymphocytes, borticulture changes in the bone marrow appear.
  
  
• II b - polycythemical with myeloid metaplasia spleen (That is, an increase in the number of all blood cells, except for lymphocytes, and the appearance of tumor growth in the spleen) is an increase in the increase in liver and spleen.
  • Proof of tumor growth in the spleen - puncture (taking a piece of fabric for a study) of the spleen and histological examination of a biopsytte (a piece of body taken by puncture).
  • Thrombosis, exhaustion, bleeding, etc. appear in the clinical picture.
  • Through the highest threat in II A and B stages are thrombosis.
  • In general blood analysis, an increase in the content of all blood cells is observed, except for lymphocytes, erythrocytes of different sizes and shapes appear, as well as immature blood cells.
  • In the bone marrow - increasing scar changes.
  
  
• III - anemic (that is, with a decrease in hemoglobin) - the outcome of the disease.
  • In the blood increases a decrease in the number of erythrocytes, platelets, leukocytes.
  • A pronounced increase in liver and spleen.
  • In bone marrow - extensive scarmers and residual formation of blood cells.
  • This stage is developing 15-20 years after the start of the disease.
  • The other tumor of blood is often joined - acute leukemia (tumor of immature blood cells) or chronic leukemia (tumor of ripe blood cells).

The reasons

The reasons the occurrence of the disease is unknown.

• A single theory explaining the occurrence of true polycythemia, as well as other hemoblastosis (blood tumors), does not exist.
• The most recognized currently is viral genetic theory.
  • According to it, special viruses (15 types of such viruses are known) are introduced into the human body and, when exposed to predisposing factors causing immunity breakdown, penetrate inside immature bone marrow cells or lymph nodes.
  • After that, immature lymph node cells instead of maturation begin to share with new cells.
  • The role of heredity in the occurrence of hemoblastosis is no doubt, since these diseases are more common in some families, as well as people with impaired structure of chromosomes (carriers of hereditary information).

Predisposing factors.

• Physical: ionizing radiation, X-ray irradiation (for example, in violation of safety technician at nuclear power plants or in the treatment of X-ray irradiation of skin tumors).
• Chemical:
  • production - varnishes, paints, etc. (they can penetrate the human body through the skin, when inhaling or deal with food and water);
  • medicinal - long-term use of gold salts (in the treatment of joint diseases, some antibiotics, etc.).

• Biological:
  • viruses;
  • intestinal infections;
  • tuberculosis (human and animal infectious disease, caused by a special type of microorganisms - mycobacteria, striking predominantly light, bones and kidney);
  • surgical interventions;
  • stresses.

Diagnostics

• Anamnese analysis of the disease and complaints (when (as long ago) there were general weakness, shortness of breath, dizziness, pain and heaviness in the left and right hypochondrium, pain in the tips of fingers, redness of the eyes, bleeding and other symptoms, with which the patient connects their occurrence).
• Anamnesis analysis of life. Does the patient have any chronic diseases if hereditary diseases are noted if the patient has bad habits, whether any drugs have taken any drugs, whether he was tumorly detected, whether it was detected with toxic substances.
• Physical inspection. The color of the skin is determined (it is possible to redness with a shiny tint, the appearance of hemorrhages). When palpation (tackling) and at percussion (climbing), an increase in liver and spleen is revealed. Pulse can be slowed down, blood pressure is elevated.
• Blood test. An increase in the number of erythrocytes (red blood cells, the rate of 4.0-5.5x10 9 g / l), the level of hemoglobin (a special compound inside erythrocytes carrying oxygen, the norm of 130-160 g / l) is determined. The color indicator (the ratio of the level of hemoglobin multiplied by 3, to the first three figures of the number of erythrocytes) usually remains normal: in the normal indicator 0.86-1.05. The number of leukocytes (white blood cells, the norm 4-9x10 9 g / l) can be elevated, normal or reduced. The number of platelets (blood plates whose gluing provides blood coagulation) first remains normal, then rises and decreases again (norm 150-400x10 9 g / l). The erythrocyte sedimentation rate (SE, a non-specific laboratory indicator, reflecting the ratio of blood proteins varieties) is usually reduced.
• Analysis of urine. It is carried out to identify concomitant pathology (diseases). Blood may appear in the urine in the development of renal bleeding or bleeding from urinary tract.
• Blood chemistry. The level of cholesterol (leaf-like substance), glucose (simple carbohydrate), creatinine (protein decomposition product), uric acid (product of decomposition of substances from cell nucleus), electrolytes (potassium, sodium, calcium) to identify concomitant organ damage. The most characteristic of true polycythemia increase in the level of uric acid due to the decay of a large number of blood cells.
• The study of the bone marrow obtained by puncture (piercing with the extraction of the internal contents) of the bone, most often the sternum (the central bone of the front surface of the chest, to which the ribs are fixed). Receives strengthening erythrocytes, platelets, leukocytes (except lymphocytes - a special type of leukocytes), as well as the development of scar tissue in the bone marrow.
• Trepanobiopia (the study of the bone marrow in its ratio with the surrounding tissues) is performed when taking a bone marrow column with a bone and periosteus, usually from the wing of the iliac bone (a person's pelvic area, located closest to the skin), with the help of special instruments - trepan. The most accurate characterizes the condition of the bone marrow.
• Ultrasound examination (ultrasound) of internal organs assesses the size of the liver and spleen, their structure for lesion by tumor cells and the presence of hemorrhages.
• Spiral computed tomography (CT) - a method based on a series of X-ray series at different depths, which allows to obtain an accurate image of the organs under study and estimate the prevalence of the tumor process.
• Magnetic resonance imaging (MRI) - a method based on building chains of water when exposed to human body of strong magnets, which allows you to obtain an exact image of the organs under study and estimate the prevalence of the tumor process.
• Consultation is also possible.

Treatment of true polycythemia

• Blooding (hemoexfusion, phlebotomy) - removal of a certain amount of blood with the medical goal. Typically remove 200-400 ml of blood, and in the elderly or in the presence of heart disease - 100 ml of blood at intervals of 2-3 days, usually on the course 3 procedures. Bloodstocks are performed in order to normalize the number of blood cells and reduce its viscosity. The procedure cannot be carried out if there are recent thrombosis (the closure of blood closet lumen with blood clots). Prior to blood consumption, drugs are prescribed, improving blood flow and reduce its coagulation.
• Extracorporeal treatments (blood purification methods outside the body using special devices). Erythrocytafferesis is applied from hardware treatment methods - removal of excess red blood cells (red blood cells) and platelets (blood plates) of blood. Performed at intervals of 5-7 days.
• Chemotherapy (the use of drugs destructive on tumor cells). Indications:
  • II B Stage,
  • increase in the number of all blood cells,
  • complications from vessels and internal organs,
  • severe patient's condition
  • ineffectiveness and poor tolerance of bloodletting.

There are special chemotherapy schemes. When the other blood tumor is connected on the III stage of the disease, chemotherapy has been carried out by an acceding blood tumor, but the efficiency is low.

• Symptomatic therapy, that is, the treatment of individual manifestations of the disease.
  • Hypotensive (reduced blood pressure drugs) are used with an increase in blood pressure. Reduced pressure when using hypotensive drugs is less significant than when bleeding. Preferably, preparations are used from the group of angiotensin-converting enzyme inhibitors (ACE), as they additionally have the ability to protect the internal organs and vessels.
  • Antihistamine (antiallergic) preparations (reduced severity of excessive manifestation of the body's protective reactions) are used to reduce the skin.
  • DeaGregants are a group of drugs that worsening blood clotting due to a violation of platelet bonding (blood plates).
  • Anticoagulants (preparations that worsening blood coagulation) are used to reduce the risk of thrombosis (closing the lumen of blood vessels with blood closures (blood clot).
  • Hemostatic (hemostatic) preparations are used in bleeding.
  • Diuretic drugs are categorically prohibited due to the high risk of developing complications due to additional thickening of blood.

• The radiation therapy (the use of ionizing irradiation for therapeutic purposes) on the region of the spleen is carried out in the IIB stage, with a pronounced increase in the spleen due to tumor growth in it.
• The transfusion of blood components is performed in the III stage of the disease, with a decrease in the content of normal blood cells.
  • The transfusion of the erythrocyte mass (erythrocytes allocated from donor blood) according to life indications (that is, if there is a patient's threat to the patient). Threat to the patient with anemia are two states:
    • anemic coma (loss of consciousness with a lack of reaction to external stimuli due to insufficient oxygen intake to the brain as a result of a significant or rapidly developed reduction in the number of erythrocytes);
    • a severe anemia (that is, the blood hemoglobin level is below 70 g / l, that is, grams of hemoglobin per liter of blood).
  • The transfusion of platelet mass is performed with a significant decrease in the number of platelets and the presence of bleeding.

Complications and consequences

• Thrombosis (closing the lumen of blood vessels with blood clots). May occur in different parts of the body. For example:
  • thrombosis of fingertips,
  • violations at the expense of eye vessel thrombosis,
  • infarction of the organs (the death of the body of the body due to the cessation of blood flow to it. Possible infarction of the spleen, muscles of the heart, kidneys, etc.).
  
  
• Thrombosis are due to impaired blood flow in small vessels by increasing blood viscosity, slowdown in blood flow, increasing the number of erythrocytes (red blood cells) and platelets (blood plates).
• Bleeding occurs at any, even small, operational interventions. Prolonged long bleeding after dental removal. Spontaneous (that is, having no known cause) bleeding rarely occurs, but they may appear, especially from the vessels of the gastrointestinal tract and hemorrhoidal nodes (extended vessels in the rear pass).
• Urolithiasis (the appearance of stones in the kidneys) and the gout (deposition of uric acid salts in various organs) are associated with the decay of a large number of blood cells.
• Without treatment, half of the patients with true polycythemia dies within 18 months after diagnosis. Therapy allows to increase lifespan, on average, for 7-15 years. The most common cause of death is thrombosis, less often - bleeding and the development of the second tumor of blood.

Prevention of true polycythemia

There are no methods for the prevention of true polycythemia.

Additionally

The incidence of true polycythemia is 0.6-1.6 new cases per 100,000 population per year.
Mostly, people aged 15 to 90 years are sick (average age of 60 years).

Basic diagnostic information is obtained in the study of peripheral blood and bone marrow. Bloodstocks, red blood cell damming, chemotherapy are used to treat polycythemia.

Policytemy

Polycythemia (Vacuise disease, Eritremia, Erythrocytosis) is a disease of the group of chronic leukemia, characterized by increased products of erythrocytes, platelets and leukocytes, an increase in OCC, splenomegaly. The disease is a rare form of leukemia: 4-5 new cases of polycythemia per 1 million population is diagnosed annually. Eritremia is developing mainly in patients of the older age group (50-60 years), somewhat more often in men. The relevance of polycythemia is due to the high risk of the development of thrombotic and hemorrhagic complications, as well as the probability of transformation into acute myeloblastic leukemia, erythromelosis, chronic myelolomicosis.

Causes of polycythemia

The development of polycithimesis is preceded by mutational changes in the polypotent stem hematopoietic cell, which gives the beginning to all three cellular lines of the bone marrow. The most often revealed mutation of the JAK2 tyrosine kinase gene with phenylalanine valine replacement in 617 positions. Sometimes there is a family incidence of Eritrea, for example, among the Jews, which may indicate in favor of genetic correlation.

With polycythemia in the bone marrow there are 2 types of erythroid blood predetermined cells: some of them behave autonomously, their proliferation is not regulated by erythropoethine; Others, as it should be, are erythropoetin-independent. It is believed that the autonomous population of cells is nothing but a mutant clone - the main substrate of polycythemia.

In the pathogenesis of Eritremia, the leading role belongs to reinforced erythropoese, the consequence of which is the absolute erythrocytosis, violation of the rheological and coagulation properties of blood, myeloid metaplasia of the spleen and liver. High blood viscosity causes a tendency to vascular thrombosis and hypoxic tissue damage, and hypervolemia causes an increased blood cell internal organs. In the finals of polycythemia there is no depletion of blood formation and myelofibrosis.

Classification of polycythemia

In hematology, 2 forms of polycythemia are distinguished - true and relative. Relative polycythemia develops at the normal level of erythrocytes and reducing the volume of plasma. This state is called stressful or false polycythemia and is not considered under this article.

True polycythemia (eritrea) by origin can be primary and secondary. The primary form is an independent myeloproliferative disease, which is based on the defeat of the myeloid sprout of blood formation. Secondary polycythemia usually develops with an increase in erythropoietin activity; This condition is a compensatory reaction to general hypoxia and may occur with chronic pulmonary pathology, "blue" vices of the heart, adrenal tumors, hemoglobinopathy, with a rise in height or smoking, etc., etc.

True polycythemia in its development passes 3 stages: the initial, deployed and terminal.

I stage (initial, low-stream) - lasts about 5 years; It proceeds asymptomatic or with minimally pronounced clinical manifestations. Characterized by moderate hypervolemia, small erythrocytosis; The size of the spleen is normal.

The stage II (eritremic, deployed) is divided into two substards:

• Ia - without myeloid transformation of the spleen. Erythrocytosis, thrombocytosis, sometimes - pancitosis; According to the myelogram - hyperplasia of all sprouts of blood formation, pronounced megakaryocytosis. The duration of the expanded stage of the eritriumlet.
• IIIL - with the presence of myeloid metaplasia of the spleen. Expressed hypervolemia, hepato and splenomegaly; In peripheral blood - pancytosis.

III Stage (anemic, post-credential, terminal). Characteristic anemia, thrombocytopenia, leukopenia, myeloid transformation of the liver and spleen, secondary myelofibrosis. Possible outcomes of polycythemia in other hemoblatosis.

Symptoms of polycythemia

Eritremia develops for a long time, gradually and can be detected by chance when studying blood. Early symptoms, such as heaviness in the head, noise in the ears, dizziness, impairment of vision, wasting limbs, sleep disorder, etc., are often "written off" for old age or related diseases.

The most characteristic feature of polycythemia is the development of the pilcitic syndrome due to pancytosis and an increase in the UNCC. Evidence of full-timeline serves teleangectation, cherry-red skin painting (especially persons, neck, hands and other open areas) and mucousse (lips, language), Schler hyperemia. A typical diagnostic sign serves as a symptom of Cooperman - the color of the solid neba remains normal, and the soft tint is becoming a stagnant cyanotic hue.

Another distinguishing symptom of polycythemia is skin itching, amplifying after water procedures and sometimes having unbearable. The erythrolealgia is also painful in the tips of the fingertips, which is accompanied by their hyperemia, also applies to the number of specific manifestations of polycythemia.

In the expanded stage of Eritremia, painful migraines may occur, pain in the bones, cardialia, arterial hypertension. 80% of patients detect moderate or severe splenomegaly; The liver increases somewhat less. Many patients with polycythemia notice increased bleeding gums, the appearance of bruises on the skin, long bleeding after the extraction of teeth.

The consequence of ineffective erythropoese during polycythemia is to increase the synthesis of uric acid and the violation of purine metabolism. This is a clinical expression in the development of so-called urate diathesis - gout, urolithiasis, renal colic.

The result of microtrombosis and trophic disturbances of the skin and mucous membranes are trophic ulcers of the leg, stomach and duodenal ulcers. The most frequent in the clinic of polycythemia complications in the form of vascular thrombosis of deep veins, mesenterical vessels, portal veins, cerebral and coronary arteries. Thrombotic complications (TELEL, ischemic stroke, myocardial infarction) are the leading causes of the death of patients with polycythemia. At the same time, along with thrombosis, patients with polycythemia are prone to hemorrhagic syndrome with the development of spontaneous bleeding of various localization (gum, nasal, from the veins of esophagus, gastrointestinal, etc.).

Diagnosis of polycythemia

Hematological changes characterizing polycythemia are determining during diagnostics. When studying blood, erythrocytosis is detected (up to 6.5-7.5x10 12 / l), increase in hemoglobin (dog / l), leukocytosis (over 12x10 9 / l), thrombocytosis (over 400x10 9 / l). Morphology of erythrocytes, as a rule, has not been changed; With increased bleeding, microcitosis can be detected. An increase in the mass of circulating erythrocytes of frozen / kg is a significant confirmation of Eritremia.

For the study of the bone marrow during polycythemia, no more informatively conducting non-sternal puncture, but trepalobiopsy. With histological examination of the bioptat, Päyelosis is detected (hyperplasia of all sprouts of blood formation), in the later stages of polycythemia - secondary myelofibrosis.

An additional laboratory tests and instrumental studies are underway to assess the risk of developing Eritremia complications - functional liver samples, general urine analysis, kidney ultrasound, limbs, EhCG, head of the head and neck vessels, FGDS, etc. In the threat of thrombohemorgic and metabolic disorders, consultations are required narrow specialists: neurologist, cardiologist, gastroenterologist, urologist.

Treatment and forecast of polycythemia

In order to normalize the volume of the BCC and reducing the risk of thrombotic complications, the first measure is bleeding. Exfusion of blood is carried out in a volume of 2-3 times a week, followed by replenishing the remote volume of blood with saline or refooliglukin. The consequence of frequent bleeding may be the development of iron deficiency anemia. Blooding during polycythemia can be successfully replaced by erythrocytafferesis, allowing to remove from blood flow only erythrocyte mass, returning the plasma.

In the case of pronounced clinical and hematological changes, the development of vascular and visceral complications resort to myelodepressive cytostatic therapy (BUsulfan, mitobronitol, cyclophosphamide, etc.). Sometimes therapy is carried out by radioactive phosphorus. To normalize the aggregate state of the blood, heparin, acetylsalicylic acid, dipyridamol under the control of the coagulogram are prescribed; with hemorrhages showing platelet transfusion; With uland diathesis - allopurinol.

Eritremia is progressive; The disease does not prone to spontaneous remission and spontaneous cure. Patients are forced to be under the supervision of the hematologist, passing gemoixfusion therapy courses. With polycythemia, high risk of thromboembolic and hemorrhagic complications. The frequency of polycythemical transformation into leukemia is 1% in patients who did not undergo chemotherapeutic treatment, and 11-15% of the cytostatic therapy.

Symptoms of true polycythemia and a forecast for life with Eritrea

Eritremia (True polycythemia, vacuise disease) - the hereditary disease of the blood system, which is predominantly in the women of the elderly.

For this pathology, malignant bone marrow hypertrophy is characterized. Most often, patients, the pathology is known as blood cancer (although such a judgment is erroneously) and leads to a progressive increase in the number of blood cells, primarily the erythrocytes (the number of other elements is also increasing). As a result of increasing their quantity, an increase in hematocrit is observed, which leads to a decrease in the rheological properties of blood, reduce the speed of blood flow by vessels, and as a result, increase the thrombosis and deterioration of tissues.

These causes lead to the fact that most tissues are experiencing oxygen starvation, which reduces their functional activity (ischemic syndrome). True polycythemia is found mainly in women. Men are sick somewhat less often, the frequency of occurrence of this pathology is approximately 3: 2.

On average, vacuise disease occurs at the age of about 40 years, and the peak of manifestation of symptoms falls on 60 - 70 years old. The hereditary predisposition to the disease is traced. In the population of Eritremia is quite rare - about 30 cases per million people.

Basic symptoms of the disease

Eritremia is an excessive blood saturation with erythrocytes, which leads to various tissue and vascular disorders. Among the most common symptoms, you can allocate:

1. Changing the color of the skin. The main reasons are stagnation and hemoglobin recovery. Due to the reduced blood flow, the erythrocytes remain longer in one place, which leads to the restoration of the hemoglobin contained in them, and as a result of this, the change in the color of the skin. Patients suffering from this disease have a characteristic appearance - a reddened face and neck of intensively cherry blossom. In addition, under the skin is clearly visible to the awaiting swollen veins. When studying the mucous membranes, it is possible to observe the characteristic symptom of Cooperman - changing the color of the soft sky with the unchanged solid color.
2. Itching This syndrome develops due to an increase in the number of immune cells with the ability to isolate specific inflammatory mediators, in particular, serotonin and histamine. Itching is enhanced after mechanical contact (most often after the shower or bath).
3. Erytroleelgia - change in the color of the distal phalanx of the fingers with the appearance of pain. This syndrome is due to the increased content of platelets in the blood, which leads to the closure of small capillaries of distal phalanx, the development of ischemic process and pain in their tissues.
4. Spleno and hepatomegaly. An increase in these organs is observed with most hematological diseases. If the patient develops eritremia, the increased concentration of blood cells can lead to an increase in blood flow in these organs, and as a result, their increase. It can be determined during palpation or with instrumental studies. Megaly syndrome is self-made after the normalization of the hemogram indicators, that is, when the blood test comes to normal.
5. Thrombose. Due to the high concentration of cells in the blood and reduction of blood flow, in the place of damage to the intima vessels, the formation of a large number of blood clots, which lead to the blockage of vessels in all parts of the body. The development of thrombosis of mesenterial, pulmonary or brain vessels is particularly dangerous. In addition, the thrombus in the small vessels of the stomach mucosa leads to a decrease in its protective properties and the appearance of gastritis and ulcers. May occur and the engine syndrome.
6. Pain. It can develop as a result of vascular disorders, for example, with refriterating endarteritis, and as a result of some metabolic disorders. In polycythemia, an increase in the level of uric acid in the blood may be observed, its deposition in the joints of the joints. In rare cases, painful syndrome is observed at percussion or the degree of flat bones containing the bone marrow (due to its hyperplasia and the stretching of the periosteum).

Among the common symptoms, if eritrium occurs, headache, dizziness, sensation in the head, noise in ears, overall weak syndrome (all symptoms are determined by a decrease in tissue oxygenation, circulatory impairment in certain parts of the body). Diagnosing are not used as mandatory criteria, as they can correspond to any systemic disease.

Stages and degrees of polycythemia

True polycythemia proceeds in three stages (stages):

• stage of initial manifestations. At this stage, the patient does not impose specific complaints. It is concerned with general weakness, increased fatigue, a sense of discomfort in the head. All these symptoms are most often charged with overwork, social and vital problems, because of which the disease itself is quite late diagnosed;
• deployed Stage (Clinical Stage). At this stage, the appearance of headaches, changes in the color of skin and mucous membranes. Pain syndrome develops quite late and indicates the nearingness of the disease;
• terminal Stage. In this stage, the lesions of the internal organs occur as much as possible due to their ischemia, violation of the function of all organism systems. A fatal outcome may come due to secondary pathology.

All stages proceed consistently, and the diagnosis of the disease (blood test) becomes informative from the clinical signs.

Diagnosis of vakes disease

To make a diagnosis, a decisive role is played by a general blood test. It has expressed erythrocytosis, an increase in hemoglobin levels and hematocrit. The most reliable is the analysis of the bone marrow punctate, in which signs of the hyperplasia of the erythroid sprout are detected, and also calculates how many cells there are also their morphological distribution.

To clarify the nature of concomitant pathology, it is recommended to conduct a biochemical analysis, which provides information on the state of the liver and kidney. With massive thrombosis, the state of blood coagulation factors is estimated by analyzing it on coagulation - coagulogram.

Other studies (ultrasound, CT, MRI) give only an indirect idea of \u200b\u200bthe state of the body and are not used in diagnosis.

Treatment of Eritremia

Despite the diversity and severity of the manifestations of vakes disease, there is relatively few methods of its treatment. It depends on what the hemogram analysis showed whether the cytological syndrome developed and which symptoms are available in the patient.

As mentioned above, the cause of the disease is the increased concentration of blood cells (especially erythrocytes), developing due to bone marrow hyperplasia. In this regard, the correct analysis of the path of development of the disease makes it possible to determine the basic principles of pathogenetic treatment, which include a decrease in the number of blood cells and the impact directly on their place of education. This is achieved by the following treatment methods:

1. Blooding. This method appeared for quite a long time, however, despite its primitiveness, it is used to this day. The essence of the procedure is the removal of blood surplus from the patient's body. This method allows to effectively reduce the pillar syndrome (full-blood), reduce the concentration of erythrocytes in the patient's blood, improve the rheological properties of its blood. The procedure is carried out several times prior to the achievement of the necessary hematological indicators (the level of hemoglobin is about 140 and the number of erythrocytes at the level of 4.5x10 ^ 12 degrees). About 300-400 ml of the patient's blood is removed in one procedure, a pre-diluted solution of refooliglukins and heparin.
2. Erythrocytheresis - Procedure aimed at the hardware removal Excess erythrocytes from the patient's body. The procedure is based on the principle of creating an artificial circle of blood circulation with, the so-called filter for red blood cells. Their excess remains on the filter membranes, and purified blood is returned to the patient's body. This method of treatment is painless, and the testimony and the necessary symptoms for it are the same as for bloodletting. However, the red blood cell damage does not cause damage to the vessels. The criterion for the effectiveness of removal of red blood cells is a normal blood test.

Such treatment should be accompanied by the appointment of antiagregative drugs such as aspirin, chief, clopidogrel or anticoagulant (heparin). The use of these drugs with one of the procedures significantly increases the effectiveness of therapy than using them separately.

It is also recommended in the treatment regimens to add some cytostatic preparations (if the cause of bone marrow hyperplasia is cancer), interferons (with the development of secondary viral complications) or hormones (mainly decxamethasone and prednisone), which makes it possible to improve the disease forecast.

Complications, consequences and forecast

All complications of the disease are due to the development of vessel thrombosis. As a result of their blockage, infarction of internal organs (heart, liver, spleen, brain) may develop, obliterates atherosclerosis (when thrombing the lower extremities affected by atherosclerotic plaques). Excess hemoglobin in the blood provokes the development of hemochromatosis, urolithiasis or gout.

All of them develop the secondary and require the elimination of the main cause - red blood cell, for the most effective cure.

As for the forecast of the disease, here much depends on the age of treatment, which methods were used, and whether they were effective.

As mentioned first, true polycythemia tends to later development. If the emergence of basic symptoms in young people is observed (aged 25 to 40 years), the disease occurs malignant, that is, the forecast is unfavorable, and the secondary complications are developing much faster. Accordingly, the later the development of the disease is observed, the more goodplace it proceeds. In the event that adequately designated drugs are used, the term and life of patients is significantly improved. Such patients may normally live with their diseases for quite a long time (up to several decades).

Responding to the question of how the outcome of Eritreia, it should be noted that it all depends on:

• what secondary processes were developed
• what are their reasons
• how long they exist
• true polycythemia was diagnosed in a timely diagnosed and necessary treatment.

Most often, due to the damage to the liver and spleen, the transition of polycythemia into the chronic form of myeloblastic leukemia is observed. The lifetime of life is preserved almost the former, and with the right selection of drugs can reach tens of years (the forecast for

Policytemy (Eritremia, Vacation Disease): Causes, Signs, Current, Therapy, Forecast

Policytemy is a disease that can be assumed only by looking at the face of the patient. And if you still have the necessary blood test, the doubts will not be left at all. In reference books, it can also be found under other names: Eritremia and vacuise disease.

Redness of the face is found quite often and always there is an explanation. In addition, it is short-term and for a long time it is delayed. Different reasons are able to cause a sudden redness of the face: fever, an increase in blood pressure, "riding" heat when climax, a recent tan, an awkward situation, and people are emotionally labile in generally blush often, even if others do not see any prerequisites for this.

Policytemy is another. Here redness is resistant, not transient, evenly distributed throughout the face. The color is unnecessarily "healthy" full-rich, bright cherry.

What kind of disease is polycythemia?

True polycythemia (eritremium, vacuise disease) refers to the group of hemoblastosis (red blood cell) or chronic leukemia with a benign flow. The disease is characterized by the growth of all three sprouts of blood formation with a significant advantage of the erythrocyte and megacariocyte, due to which there is an increase of not only the number of red blood tauros - erythrocyteBut the rest of the blood cells that originate from these sprouts, where the source of the tumor process is the affected predecessor cells of myelopoose. It is they who begin an uncontrolled proliferation and differentiation into mature form of red blood cells.

Most of all in such conditions suffer unripe red blood tales, which have hypersensitivity to erythropoietin even in small doses. In polycythemia, the growth of the leukocytes of the granulocytic series is also observed (first of all, laughter and neutrophils) and platelets. The lymphoid cells, which include lymphocytes, the pathological process does not concern, as they originate from another sprout and have a different way of breeding and maturation.

Cancer or not cancer?

Eritremia - not to say that it is completely different, but on the town of 25 thousand population a couple of people will be found, with the men years of 60 or so so this disease "loves" for some reason more, although to meet with such pathology can face anyone age. True, for newborn and younger children, true polycythemia is absolutely not characteristic, so if Eritremia is found in a child, then, most likely, it will be secondary character and be a symptom and consequence of another disease (toxic dyspepsia, stress-erythrocytosis).

For many people related to leukemia (and no matter: acute or chronic) disease, first of all, is associated with blood cancer. Is it interesting to understand: cancer is it or not? In this case, it is more expedient, it will be more suitable for the malignancy or benignness of true polycythemia to determine the border between "good" and "evil". But, since the word "cancer" denote tumors from epithelial fabrics, in this case, this term is inappropriate, because this tumor comes from hugness fabric.

Vakes disease belongs to malignant tumorsBut at the same time is characterized by high cell differentiation. The course of the disease is long and chronic, for the time being qualifiable as benign. However, such a flow can only last until a certain moment and then with proper and timely treatment, but after a date, when significant changes in erythropoese occur, the disease turns into an acute form and acquires more "evil" features and manifestations. This is such a true polycythemia, the forecast of which will depend fully on how quickly its progression will occur.

Why do sprouts grow wrong?

Any patient suffering from Eritremia, sooner or later asks for: "Why did this" rode happened "with me?". Finding the cause of many pathological conditions is usually useful and gives certain results, increases the effectiveness of treatment and contributes to recovery. But not in the case of polycythemia.

The causes of the disease can only be assumed, but not to assert unambiguously. Only one hook can be for a doctor when finding out the origin of the disease - genetic anomalies. However, the pathological gene has not yet been found, so the accurate localization of the defect has not yet been determined. True, assumptions that the vacuise disease can be associated with trisomy 8 and 9 pairs (47 chromosomes) or another disorder of the chromosomal apparatus, for example, the loss of the plot (deletion) of the long shoulder C5, C20, but this is as long as the guesses, although built on conclusions of scientific surveys.

Complaints and clinical picture

If there is nothing to say about the causes of polycythemia, then you can talk about clinical manifestations for a long time and a lot. They are bright and diverse, since already with 2 degrees of development of the disease in the process turn out to be drawn literally all organs. Subjective patient sensations are general:

• Weakness and constant feeling of fatigue;
• A significant reduction in performance;
• Increased sweating;
• Headaches and dizziness;
• Noticeable reduction in memory;
• Summary and auditory disorders (reduction).

Complaints peculiar to this disease and characterized by:

• Acute burning pain in the fingers of the hands and legs (the vessels are clogged with platelets and erythrocytes, which form small aggregates there);
• Soreness, however, not so burning, in the upper and lower limbs;
• Itching body (a consequence of thrombosis), the intensity of which is noticeably intensified after the shower and hot bath;
• Periodic appearance of uri type of urban.

It's obvious that cause all these complaints disruption of microcirculation.

redness of the skin during polycythemia

As the disease further develops, more and more new symptoms are formed:

1. Hyperemia of the skin and mucous meal due to the expansion of capillaries;
2. Pain in the heart, resembling angina;
3. Painful sensations in the left hypochondrium caused by overload and increasing spleen due to the accumulation and destruction of platelets and erythrocytes (it is a kind of depot for these cells);
4. Increase in liver and spleen;
5. Ulcerative ulcer of the stomach and 12 pans;
6. Dysuria (urination act) and pain in the lumbar region due to the development of uricinal diathesis caused a shift in blood buffer systems;
7. Pains in the bones and joints as a result hyperplasia (redundancy) bone marrow;
8. Gout;
9. Manifestations of hemorrhagic nature: bleeding (nose, gum, intestinal) and skin hemorrhages;
10. Injections of the vessels of conjunctiva, which is why the eyes of such patients are called "rabbit eyes";
11. Telegangectasi;
12. A tendency to vein thrombosis and arteries;
13. Varicose veins of the shin;
14. Thrombophlebitis;
15. Coronary vessels are possible with the development of myocardial infarction;
16. Intermittent chromotype, which can end with gangrena;
17. Arterial hypertension (almost 50% of patients), generating a tendency to strokes and infarction;
18. Breathing damage due to immunity disorderswhich cannot adequately respond to infectious agents that cause inflammatory processes. In this case, the red blood cells begin to behave like suppressors and suppress the immunological response to viruses and tumors. In addition, in the blood, they are in abnormally high quantities, which further aggravates the state of the immune system;
19. The kidneys and urinary paths suffer, so patients have a tendency to pyelonephritis, urolithiasis;
20. It does not remain aside from the events in the body and the central nervous system, when involving it in the pathological process, symptoms of brainwater disorders appear, ischemic stroke (with thrombosis), hemorrhage (less often), implantation, memory violation, many-lane disorders.

From the asymptomatic period to the terminal stage

Due to the fact that for polycythemia in the first stages, the asymptomatic flow is characterized, the above manifestations arise not in one day, but accumulate gradually and for a long time, in the development of the disease it is customary to distinguish 3 stages.

Initial stage. The patient's condition is satisfactory, the symptoms are moderately pronounced, the duration of the stage is about 5 years.

Stage of deployed clinical manifestations. It takes place in two stages:

II A - flows without myeloid metaplasia of the spleen, the subjective and objective symptoms of Eritremia are present, the duration of the campaign period;

II B - a myeloid spleen metaplasia appears. This stage is characteristic of a clear picture of the disease, the symptoms are pronounced, the liver and spleen are significantly increased.

Terminal stage having all signs of a malignant process. Sick complaints are diverse, "everything hurts, everything is wrong." At this stage, the cells lose the ability to differentiate than create a substrate for leukemia, which replaces chronic eritrium, or rather, it goes into acute leukemia.

The terminal stage is particularly severe (hemorrhagic syndrome, separation of the spleen, infectious-inflammatory processes that cannot be treated due to deep immunodeficiency). It usually ends with a fatal outcome.

Thus, life expectancy during polycythemia to strike, which may be well, especially if you consider that the disease can be overtaken after 60. This means that there is some prospect to live to 80 years. However, the forecast of the disease is still more dependent on its outcome, that is, from which the form of leukemia is transformed on the III stage (chronic myelolomicosis, myelofibrosis, acute leukemia).

Diagnosis of vakes disease

The diagnosis of true polycythemia is primarily based on laboratory studies with the definition of the following indicators:

• A common blood test, in which a significant increase in erythrocytes (6.0-12.0 x / l), hemoglobin (g / l), hematocrit (the ratio of plasma and red blood) is noted. The number of platelets can reach levels of 10 9 / l, while they can significantly increase in size, and leukocytes - up to 9.0-15.0 x 10 9 / l (due to chopsticks and neutrophils). ESO with true polycythemia is always reduced and can reach zero.

Morphologically erythrocytes do not always change and often remain normal, but in some cases, with eritrium, you can observe anisocytosis (Erythrocytes of different sizes). The degree of gravity and prognosis of the disease in polycythemia in general blood test indicate platelets (than them are more, the heavier the course of the disease);

• Tank (biochemical blood test) with the determination of the level of alkaline phosphatase and uric acid. For Eritremia, the latter accumulation is very characteristic, which indicates the development of the gout (a consequence of the disease of the vakes);
• Radiological research using radioactive chromium helps determine the increase in circulating erythrocytes;
• Sternal puncture (bone marrowing of the bone marrow from the sternum) with subsequent cytological diagnostics. In the preparation - hyperplasia of all three sprouts with a significant predominance of red and megacariocyte;
• Trepanobiopia (histological examination of the material taken from the iliac bone) is the most informative method that allows the most reliably to identify the main sign of the disease - three-rigid hyperplazia.

In addition to hematological indicators, to establish the diagnosis of true polycythemia of the patient, they are sent to an ultrasound study (ultrasound) organs of the abdominal cavity (increase in liver and spleen).

So, the diagnosis is set ... What's next?

And then the patient is waiting for treatment in the hematological profile department, where clinical manifestations, hematological indicators and the stage of the disease determine the tactics. In therapeutic measures, Eritremia usually includes:

1. Blooding, which allows to reduce the amount of red blood cells up to 4.5-5.0 x / l and Hb (hemoglobin) to 150 g / l. To do this, with an interval of 1-2 days, 50 ml of blood is taken as long as the amount of erythrocytes and HB will not fall. The hematologists are sometimes replaced by the hematirts to be replaced by erythrocyt farresis, when red blood is separated by centrifuging or separation by the centrifugation or separation method, and the plasma returns to the patient;
2. Cytostatic therapy (myelosan, imyphos, hydroxyuropean, hydroxycarbamide);
3. Antiagregants (aspirin, dipyridamol), which, however, require caution in application. Thus, acetylsalicylic acid is able to increase the manifestation of hemorrhagic syndrome and cause internal bleeding in the presence of a patient with a gastric ulcer or 12 reps;
4. Interferon-α2B, successfully used with cytostatics and increases their effectiveness.

Eritremia treatment scheme is written by a doctor for each case individually, so our task is only briefly introducing a reader with drugs used to treat vakes disease.

Food, diet and folk remedies

A considerable role in the treatment of polycythemia is given to the labor regime (reduction of physical exertion), recreation and nutrition. In the initial stage of the disease, when symptoms are not yet expressed or poorly manifested, the patient is prescribed table No. 15 (general), however, with some reservations. The patient does not recommend using food reinforcing products (liver, for example) and offer to revise the diet, giving preference to dairy and vegetation products.

In the second stage of the disease, the patient is appointed table No. 6, which corresponds to a diet when gouging and limits or altogether excludes fish and meat dishes, legumes and sorrel. After recovering from the hospital, the patient must adhere to these by the doctor of recommendations and with outpatient observation or treatment.

Question: "Is it possible to treat folk remedies?" Sounds with the same frequency for all diseases. Eritremia is no exception. However, as already seen, the course of the disease and the life expectancy of the patient is entirely dependent on the timely treatment of the treatment, the purpose of which is to achieve a long and resistant remission and pulling out the third stage for the longest time.

During the period of the clutch of the pathological process, the patient should still remember that the disease can return at any time, so he must discuss his life out of exacerbation with the attending physician who has been observed, to periodically take tests and survey.

Treatment by people's blood diseases should not be generalized and, if there are many recipes for increasing the level of hemoglobin or for blood liquefaction, it does not mean at all that they are suitable for the treatment of polycythemia, from which, in general, the medicinal herbs has not yet been found. Vaca disease is a thin and in order to control the bone marrow function and thus influence the blood formation system, you need to have objective data, which can evaluate a person who has certain knowledge, that is, the attending physician.

In conclusion, I would like to say to readers a few words about relative eritremia, to confuse with the true one cannot, since the relative red blood cells may arise against the background of many somatic diseases and successfully end when the disease is healing. In addition, erythrocytosis as a symptom can accompany long-term vomiting, diarrhea, burn disease and hyperhydrosis. In such cases, erythrocytosis is temporary and is associated, first of all, with dehydration of the body, when the amount of circulating plasma consisting by 90% of the water is reduced.

Forecast for true polycythemia

Among blood diseases there are many such that cause a decrease in different elements - erythrocytes, leukocytes, thrombocytees. But with some pathologies, on the contrary, there is an uncontrolled increase in the number of blood cells. A condition in which a chronic increase in the number of erythrocytes is observed, and other pathological changes occur, the name "True polycythemia" is called.

Features of the disease

Primary (true) polycythemia - blood disease from the leukemia group arising idiopatically (without visible causes) flows for a long time (chronically) and is characterized by an increase in the number of erythrocytes, an increase in blood hematocrit and blood viscosity. Synonyms of pathology names - Vacan-Osler's disease, Eritremia, primary red blood cell. The effects of red blood cell and thickening of blood with this myeloproliferative disease can be serious and relate to the risk of developing thrombosis, an increase in size and disruption of the work of the spleen, the increase in the volume of circulating blood, etc.

Eritremia is considered a malignant tumor process, which is due to reinforced proliferation (hyperplasia) of bone marrow cells. A particularly strong pathological process covers the erythrobist sprout - part of the bone marrow consisting of erythroblasts and normoblasts. The pathogenesis of the main manifestations is associated with the appearance of a huge amount of erythrocytes in the blood, as well as with some increase in the number of platelets and neutrophils (neutrophilic leukocytes). Blood cells at the same time morphologically normal, but their number is anomalous. As a result, blood viscosity increases and the amount of blood in circulating blood flow. The result becomes a slower flow of blood, the formation of thrombus, violation of local blood supply to the tissues and their hypoxia.

If initially the patient is most often observed primary red blood cells, that is, only the number of erythrocytes increases, then other blood cells begin to cover further changes. Extramedullary blood formation (the pathological formation of blood outside the bone marrow) occurs in the organs of the peritoneum - in the liver and spleen, where the part of the erythropoese is also localized - the erythrocyte formation process. At the late stage of the disease, the life cycle of red blood cells is shortened, anemia, thrombocytopenia, myelofibrosis can develop, and leukocyte precursor cells and erythrocytes are in total bloodstream, not mature. In about 10% of cases, pathology flows into acute leukemia.

The study and first description of the red blood cells was made in 1892 by the Vakesom, and in 1903 the scientist Osler suggested that the cause of the disease is a disruption of the operation of the bone marrow. True polycythemia is somewhat more common than other similar pathologies, but still is quite rare. It is diagnosed about 5 people per year per million population. Most often, the disease is celebrated in people over 50 years old, the average age of detection is 60 years. In children, such a diagnosis is made very rarely, mainly after 12 years. On average, only 5% of the ill people are under 40 years old. Men sick such pathology more often than women. In the overall structure of chronic myeloproliferative diseases, true polycythemia ranks 4th. Sometimes it is inherited, so family cases are found.

Causes of pathology

The primary form of the disease is considered hereditary, transmitted on autosomal-recessive type. In this case, it is often referred to as "Family Policytemy". But most often Eritremia is a secondary state, representing one of the manifestations of the general pathological process. Exact reasons are not established, but there are several theories about the appearance of true polycythemia. Thus, there is a link to the development of a disease with the transformation of stem cells, when a tyrosine kinase mutation occurs in true polycythemia more often than with other blood diseases.

Cell studies during Eritremia revealed in many patients the clone origin of pathology, since in leukocytes, platelets, they have been identified by the same enzyme in leukocytes. The clone theory confirms the conducted cytological studies on the karyotype of groups of chromosomes, where various defects were identified, similar to different patients. There is also a viral-genetic theory, according to which up to 15 types of viruses can be introduced into the body and, with the participation of a number of provoking factors, lead to a failure in the work of the bone marrow. They penetrate the predecessors of blood cells, which then instead of normal maturation begin to share and form new erythrocytes and other cells.

As for the risk factors for the development of true polycythemia, then, presumably, they may be as follows:

• lung diseases;
• long stay at high height above sea level;
• hy hypoventilation syndromes;
• different hemoglobinopathy;
• long smoking experience;
• bone marrow tumors, blood;
• hemokoncentration with a debt of diuretic reception;
• burns of a large part of the body;
• strong stress;
• diarrhea;
• radiation by x-ray, radiation;
• poisoning of pairs of chemicals, hitting them through the skin;
• admission of poisoning substances in the gastrointestinal tract;
• treatment of gold salts;
• tuberculosis running form;
• serious surgical interventions;
• "Blue" heart defects;
• pathology of kidneys - hydronephrosis, stenosis of the kidney arteries.

Thus, the main cause of secondary erythrocytosis is all states that such or other provoke hypoxia tissues, stress for the body or its intoxication. In addition, oncological processes, endocrine pathologies, liver disease may have a large influence on the brain and developing them.

Classification of true polycythemia

The disease is classified in the following steps:

1. The first, or initial stage. It may continue for more than 5 years, represents the development of the pilor syndrome, that is, enhancing the blood flow of the organs. At this stage, the symptoms can be expressed moderately, there is no complications. The overall blood test reflects a minor increase in the number of erythrocytes, the bone marrow puncture shows the enhancement of erythropoese or products of all major elements of blood, with the exception of lymphocytes.
2. Second - A stage, or polycythmical stage. Duration - from 5 to 15 years. Pletic syndrome is more pronounced, an increase in spleen, liver (hematopoietic organs) is observed, thrombosis in veins and arteries is often registered. Tumor growth in the organs of the peritoneum is not observed. If this stage is completed with a decrease in platelets - thrombocytopenia, then various bleeding can occur in the patient. Frequent hemorrhages cause a lack of iron in the body. A general blood test reflects an increase in red blood cells, platelets, leukocytes, at a launched flow - a decrease in platelets. In myelogram, the imminent formation of most blood cells is noted (exception - lymphocytes), scar brain changes are formed.
3. The second stage, or the polycythemical stage with the myeloid metaplasia of the organ - spleen. The patient continues to increase the size of the spleen, and often liver. When puncture spleen, tumor growth is detected. There are frequent thrombosis, intermitted with bleeding. In general analysis, there is an even greater increase in the number of erythrocytes, leukocytes, there are erythrocytes of different sizes, form, there are immature precursors of all blood cells. In the bone marrow the number of scar changes is increasing.
4. Third, or anemian stage. It is the outcome of the disease, in which the depletion of blood cells is depleted. The number of erythrocytes, leukocytes, as well as platelets, is much reduced, the liver and spleen are increased with myeloid metaplasia, extensive scar changes occur in the bone marrow. A person gets disability, most often due to the consequences of thrombosis or the addition of acute leukemia, myelofibrosis, hypoplasia of blood formation or chronic myelolecosis. This stage is registered roughly from the moment of the development of pathology.

Symptoms of manifestation

Often, this pathology proceeds asymptomatic, but only at the initial stages. Later, the disease in the patient, one or another manifests itself, while specific symptoms can be diverse. Basically, the symptom complex includes such basic signs:

1. Changing skin shade, veins expansion. Most often, in the area of \u200b\u200bthe neck in an adult, they begin to strongly shone the veins, their drawing becomes stronger due to swelling, overflowing blood. But the most apparent skin signs are: the skin color becomes dark red, literally cherry. Most of all this is noticeable in the area of \u200b\u200bthe neck, hands, the face, which is associated with the overflow of the blood of the subcutaneous arteries. At the same time, many patients mistakenly think that blood pressure rises against the background of hypertension, and therefore often continue to take drugs from pressure and do not turn to the doctor. With a careful attitude to health, it can be noted that the lips and the tongue also changed their shade, became red-blue. Blood and eye vessels are poured, their full-row leads to hyperemia sclera and conjunctivities of organs of vision. The solid sky remains the same color, but the soft sky also becomes brighter, burgundy.
2. Itching skin. All described changes from the skin around at half past cases are complemented by strong discomfort and itching. This feature is very characteristic of Eritremia, both for primary and secondary. Since after the adoption of water procedures, histamine is released in patients, as well as prostaglandins, it can become even more pronounced after the bath or soul.
3. Pain in the limbs. Many people develop obliterating endarteritic, as a result of which persistent and severe painful sensations appear in the legs. They can enhance under load, long walking, in the evening, at first are often perceived as a symptom of fatigue in an elderly person. Pains are observed during palpation and the degree of flat bones, which reflects the process of hyperplasia and scar changes in the bone marrow. The following type of pain in a person with true polycythemia is a stubborn burning pain in the field of large and small foot joints, which resemble gouty pain and are due to the same reason as the gap is an increase in the level of uric acid. Another type of pain is the strongest, poorly portable pain in the fingers of the hands, the legs, in which the skin becomes bluish-red, the blue spots appear on it. These pains are due to the growth in the number of platelets and the advent of the microtrombosis of capillaries.
4. Splenomegaly. The growth of spleen in size is observed in almost every person with true polycythemia, but at different stages of the disease. This happens because of the increase in the filling of the spleen with blood and the development of myeloproliferative phenomena. Somewhat less often, but still occurs and a strong increase in the size of the liver - hepatomegaly.
5. Peptic disease. Approximately every tenth person with a vacuiz-washer disease occurs in a small intestine (more often in the duodenalist) and in the stomach. This is due to the activation of Helicobacter Pylori bacteria, as well as the development of microtrombosis in the zhtskaya area.
6. Thrombosis and bleeding. Almost all of all sick at a certain stage appears a tendency to thrombosis, and even until recently, patients died from such complications at an early stage of the disease. Now the current current treatment can prevent the appearance of blood clots in the brain, spleen, legs that threaten embolism and death. Increased blood viscosity characterizes true polycythemia at the initial stages, and later against the background of the depletion of the platelet education system, bleeding is developing - it is observed in the area of \u200b\u200bgums, nose, uterus, gastrointestinal tract.

There are other signs of true polycythemia, which a person may complain, but they are minor specific and can be inherent in different pathologies:

• fatigue;
• heads;
• noises in the ears;
• nausea;
• dizziness;
• the feeling of ripples in whiskeys, ears;
• decline in appetite, performance;
• the appearance of "flies" before the eyes;
• other violations - field loss, the fall in visual acuity;
• shortness of breath;
• increased blood pressure;
• inexplicable weight loss;
• long subfebrile;
• insomnia;
• numbness, pinching of fingers;
• epileptiform seizures and paralysis (rarely).

In general, the disease is characterized by a long and sometimes benign course, especially when conducting adequate treatment. But some people, especially those who do not receive therapy, may be early emergence of various consequences of true polycythemia.

Possible complications

Most often complications are associated with thrombosis and embolines of veins and vessels of spleen, liver, legs, brain, other areas of the body. This leads to different consequences depending on the magnitude of the thrombus, the lesion zone. Transient ischemic attacks, strokes, thrombophlebitis and phlebommboosis of surface and deep veins, clogging of retinal vessels and blindness, infarction of domestic organs, myocardial infarction, blockage.

In the lowest stages of pathology, stones in the kidneys (urolithiasis), gout, nephrosclerosis, liver cirrhosis appear. Probably the emergence of complications due to the bleeding of tissues - bleeding from the ulcer of the gastrointestinal tract, anemia. From the heart, besides myocardial infarction, the appearance of signs of myocardiosclerosis, heart failure. There is also the likelihood of the transition of true polycythemia in acute leukemia, chronic leukemia and other oncopathology.

Diagnosis

To diagnose this disease is not easy, especially, in the absence of a characteristic clinical picture and in the presence of only common symptoms. Nevertheless, the totality of these hematological and biochemical analyzes, as well as some distinguishing features of the appearance of the patient, with his complaints, will help the doctor to establish the cause of the changes.

Basic in order to establish the diagnosis of true polycythemia are the indicators of the overall analysis of blood - the number of erythrocytes and hematocrit. In men, they can suspect the development of this disease, if the number of erythrocytes is more than 5.7 * 10 * 9 / l, hemoglobin is more than 177 g / l, the hematocrit was above 52%. In women, exceeding indicators noted if they constitute more than 5.2 * 10 * 9 / l, 172 g / l, 48-50%, respectively. These figures are characteristic of the early stages of pathology, and as they have been developed, they become even higher. In addition, an assessment of the mass of circulating erythrocytes, which is normal for men will be up to 36 ml / kg, in women - up to 32 ml / kg.

Other blood indicators (biochemistry, general analysis and other analyzes), which in a complex with disorders described and in combination with each other reflect the picture of the development of primary or secondary red blood cells:

1. Moderate or pronounced thrombocytosis (above 400 * 10 * 9 l), neutrophilic leukocytosis (above 12 * 10 * 9 l) with the presence of an increased amount of basophils and eosinophils.
2. Increasing the number of reticulocytes.
3. The appearance in the blood of myelocytes, metamielocytes.
4. Increase blood viscosity by%.
5. Strong decline in SE.
6. An increase in the mass of circulating erythrocytes.
7. Increased alkaline phosphatase, vitamin B12 in serum.
8. The increase in the amount of uric acid in serum.
9. Blood saturation in oxygen arteries above 92%.
10. The appearance of the colonies of red blood cells in the test tube.
11. Reduced erythropoietin indicator.
12. Changing the color indicator of less than 1.

At the stage of myelofibrosis, hemoglobin indicators, erythrocytes may come back, but at the same time the number of leukocytes increases, they appear immature forms, the presence of erythroblasts is diagnosed. As for the myelogram, which is obtained by puncturing the bone marrow, such changes are detected in it:

• reducing the presence of fat inclusions;
• enhance the erythroblasts, normoblasts;
• hyperplasia of Mielopoese sprouts.

There are other criteria for which the doctor can conclude about the changes derived for the true polycythemia:

1. Hepatosplegegaly.
2. Template to thrombosis.
3. Increased sweating in combination with weight loss, weakness.
4. The presence of gene anomalies, if genetic testing was carried out when it comes to primary eritrium.
5. Increase the average number of circulating blood.

All the criteria described above, except for the three main, considered large, are small. As for large diagnosis criteria, this is an increase in the mass of circulating erythrocytes, splenomegaly, the oversaturation of arterial blood oxygen. To establish a diagnosis, it is usually enough to have three indicated large criteria that are combined with two or three small. Differential diagnosis is carried out by a hematologist between states that are accompanied by erythrocytosis - heart defects, tuberculosis, tumors, etc.

Treatment methods

The earlier the person appealed to help, the more effective may be therapy. In the third stage, or with the layering of another tumor process, symptomatic therapy is carried out on Eritrea in combination with the treatment of chemotherapy. Chemotherapeutic treatment can be recommended on other stages of the disease, but the body does not always give it an adequate answer. Of the symptomatic means that improve the quality of life, these are applied:

1. Preparations against high blood pressure, mainly from the group of ACE inhibitors.
2. Antihistamine drugs from itching, skin irritation, other allergic reactions.
3. Antiagregants and anticoagulants for blood dilution with a leakage to thrombosis.
4. Local and systemic hemostatic drugs when bleeding fabrics.
5. Medicines to reduce uric acid indicator.

Treatment methods of true polycythemia may be:

1. Blooding, or removal of a small part of blood from blood flow (phlebotomy). As a rule, they are made in bulk (according to indications) and a break in 3-4 days by a course of several sessions. Blood after such manipulations becomes more liquid, but it is impossible to make them in the presence of thrombas in a recent history. Before treatment with bleeding, the patient is introduced a solution of refooliglucin, as well as heparin.
2. Erythrocytafarez. Used to clean blood from excess red blood cells, as well as platelet. Such sessions are made once a week.
3. Chemotherapy. Used, as a rule, when achieving the disease of the tumor stage - the second B. other testimony to carry out chemotherapy are the presence of complications from the organs of the peritoneum, the overall part of the person, the increase in the number of all blood elements. Cyticostatics, antimetabolites, alkylating preparations, biological preparations are used for chemotherapy, or cytine deuctive therapy. Most often prescribed drugs leykeeran, hydroxymeurum, myelosan, recombinant interferon.
4. Treatment of iron deficiency with androgens, erythropoietin, which are most often used in a complex with glucocorticosteroids.
5. Radiation therapy. Used to irradiate the area of \u200b\u200bthe spleen and termination of the cancer process in it, it is used with a strong increase in the body in sizes.
6. Transfusion of erythrocyte mass of purified red blood cells. Used with severe anemia up to the state of the coma. If thrombocytopenia increases at the final stages of true polycythemia, it may be necessary to transfay the mass of platelets from the donor.

Bone marrow transplantation with such a disease, as eritremia, often leads to adverse results, it is rarely applied. In some cases, the execution of splenectomy is shown, but in the development of acute leukemia, such an operation is not made even with pronounced splenomegaly.

Features of pregnant women

During pregnancy, this pathology rarely occurs. However, in the presence of predisposition (hereditary or from secondary factors), pregnancy, childbirth and abortions may become a starting mechanism for the development of true polycythemia. Pregnancy always worsens the course of this disease, and its outcome may be more serious than outside gestation. Nevertheless, in 50% of cases, pregnancy ends with prosperous childbirth. The rest of the half falls on miscarriages, delay in the development, anomalies of the body of the fetus.

Treatment of disease in pregnant women should be difficult. Most of the drugs are strictly contraindicated, as it has a pronounced teratogenic property. Therefore, during pregnancy, predominantly, therapy is performed by bloodstand and, if necessary, glucocorticosteroids. To prevent complications and early detection of the disease, pregnant women should regularly conduct blood tests according to the graphics indicated by a gynecologist observing obstetrician.

What can not do

It is categorically impossible to use diuretics that additionally thicken blood. Also, in our time, the use of radioactive phosphorus preparations, which seriously oppress myelopoese and often lead to the development of leukemia. Also cannot be saved the same power system: the diet must change. Under the ban all the food that enhances the blood formation, for example, the liver. It is better to form a diet as milky-plant, and from excess meat to refuse.

The patient can not be overloaded the body, engage in heavy sports, ignore regular rest. It is possible to use the treatment with folk remedies, but only after a thorough study of all the funds by a doctor in composition to prevent the enhancement of erythrocyte production. Most often use symptomatic therapy for removing uric acid, reduce pain and skin itching, etc.

Prevention and forecast

Methods of prevention have not yet been developed. Forecast for life varies depending on the severity of the course of the disease. Without treatment up to a third of the patients, dies during the first 5 years from the date of diagnosis. If you carry out full therapy, you can extend the life of a person and more. Most often, the cause of death is thrombosis, and only sometimes people die from blood cancer (leukemia) or heavy bleeding.

Polycythemia is a chronic disease in which the content of red blood cells (red blood cells) in the blood increases. Also, with such pathology, 70% of patients change the amount of platelets and leukocytes in the most side.

NEUCH is not highly prevalent - annually one million people register no more than five cases. Most often, polycythemia disease develops in middle and elderly people. According to statistics, male representatives suffer from this pathology five times more often than women. Today we consider in more detail such a state as polycythemia, symptoms and treatment of pathology will be described below.

Causes of disease development

Polycythemia is not a malignant disease. To date, the exact causes of illness are unknown. It is believed that the development of pathology causes mutation of a special bone marrow enzyme. Gene changes lead to excessive division and growth of all blood cells, and in particular erythrocytes.

Classification of the disease

Allocate two groups of illness:

True polycythemia, or a vacuise disease, which in turn is divided into primary (that is, acts as an independent disease) and secondary (secondary polycythemia develops due to chronic lung diseases, tumors, hydronephrosis, lifting height).

Relative polycythemia (stressful or false) - with this state, the level of erythrocytes remains within the normal range.

Polycythemia: Symptoms of illness

Very often the disease proceeds asymptomatic. Sometimes, as a result of a survey for completely different reasons, true polycythemia may be accidentally revealed. Symptoms that should be paid attention to, consider further.

Expansion of subcutaneous veins

With polycythemia on the skin, most often in the neck area, extended subcutaneous veins appear. With such pathology, the skin becomes a reddish-cherry shade, especially this is noticeable in open areas of the body, brushes, face. The mucous membrane of the lips and the language has a blue-red color, the eyes of the eyes seemed to be poured.

It causes such changes with blood rich with erythrocytes, all surface vessels and slowing its rheological properties (movement speed), as a result of which the main part of the hemoglobin (red pigment) goes into a restored form (that is, it is subjected to chemical changes) and changes the color.

Skin itch

Practically, half of patients suffering from polycythemia occurs heavy skin itching, especially manifested after the adoption of a warm bath. Such a phenomenon acts as a specific feature of true polycythemia. Itching arises due to the emission of active substances into the blood, in particular histamine, which is capable of expanding the skin capillaries, which leads to an increase in blood circulation in them and the appearance of specific sensations.

Erythrolalalagia

Such a phenomenon is characterized by short-term strongest pains in the field of fingertips. It provokes them to increase the level of platelets in small vessels of the brush, as a result, numerous microtromb are formed, blocking arterioles and blocking blood flow to the tissues of the fingers. External signs of such a state is redness and appearance of blue spots on the skin cover. It is recommended to take aspirin.

Splenomegaly (increasing spleen)

In addition to the spleen, the liver can change, or rather, its size. These bodies are directly involved in the formation and destruction of blood cells. The increase in the concentration of the latter leads to an increase in the size of the liver and spleen.

and stomach

Such a serious surgical pathology develops due to thrombosis of small vessels of the mucous membrane of the digestive tract. The result of acute is the death (necrosis) of the area of \u200b\u200bthe body wall and the formation of an ulcerative defect in its place. In addition, the stomach stability is reduced to Helicobacter (microorganism, causing gastritis and ulcers).

Clomes in large vessels

More susceptible to such pathology of veins of lower extremities. From the wall of the vessel, maybe, bypassing the heart, penetrate into a small circle of blood circulation (lungs) and provoke TEL (pulmonal artery thromboembolism) - a state incompatible with life.

Blooding the gums

Despite the fact that the number of platelets in peripheral blood changes and its coagulation increases, the leaks may occur during polycythemia.

Gout

With an increase in the level of uric acid, its salts are deposited in various joints and provoke a sharp pain syndrome.

• Pain in the limbs. Such a symptom causes damage to the arteries of the legs, their narrowing and, as a result, circulatory disruption. This pathology has the name "Supporting ENDARTERIT"
• Pain in flat bones. The increased activity of the bone marrow (location of blood cells) provokes the sensitivity of flat bones to mechanical effects.

Deterioration of the general condition of the body

With such a disease, like polycythemia, symptoms may be similar to the signs of other pathologies (for example, anemia): headaches, constant fatigue, ears, dizziness, mugs in front of the eyes, shortness of breath, increasing the viscosity properties of blood activates the compensatory reaction of the vessels As a result, there is an increase in blood pressure. With this pathology, complications are often observed in the form of heart failure and microcardiosclerosis (substitution of the muscle tissue of the heart of the connective, filling defect, but not performing the necessary functions).

Diagnostics

Reveal polycythemia based on the results of a general blood test, which detects:

increased amount of red cells from 6.5 to 7.5.10 ^ 12 / l;

increased level of hemoglobin - up to 240 g / l;

the total volume of erythrocytes (RO) exceeds 52%.

Since the number of erythrocytes cannot be calculated based on the measurements of the above values, radionuclide diagnosis is used for measurement. If the mass of red blood cells exceeds 36 ml / kg in men and 32 ml / kg in women, it reliably testifies to the presence of vakes disease.

When polycithhemia, the morphology of red blood cells persists, that is, they do not change their normal shape and sizes. However, in the development of anemia as a result of increased bleeding or frequent bleeding, microcitosis is observed (decrease in red blood cells).

Policytemy: Treatment

A good therapeutic effect has bloodletting. It is recommended to remove 200-300 ml of blood weekly until the OOE level drops to the desired value. If there are contraindications for blood consideration, it is possible to restore the percentage of erythrocytes with the help of blood dilution, adding liquid part to it (high molecular weight solutions intravenously).

It should be borne in mind that quite often bloodletting leads to the development of iron deficiency anemia, in which the corresponding symptoms and an increase in platelet content are observed.

With such a notem, as true polycythemia, treatment implies compliance with a certain diet. It is recommended to limit the use of meat and fish products, as they are contained in a high amount of protein, which is actively stimulating the activity of blood formation organs. Also should be abandoned by oily food. Cholesterol contributes to the development of atherosclerosis, as a result of which thrombus arise, which are in large quantities and so form in people suffering from polycythemia.

Also, if the diagnosis of "polycythemia" is diagnosed, treatment may include chemotherapy. Apply it with increased thrombocytosis and severe itching. As a rule, this is a "cytiratement agent" (preparation "Hydroxycarbamide").

Until recently, the injection of radioactive isotopes (usually phosphor-32) was used to suppress the bone marrow (usually phosphorus). Today, increasingly refuses to treat such treatment, due to the high speed of leukemic transformation.

Therapy also includes interferon injections, an Anagrelide preparation is used in the treatment of secondary thrombocytosis.

With this pathology, it is very rarely carried out as polycythemia is a disease that is not fatal, provided, of course, adequate treatment and continuous control.

Polycythemia in newborns

Polycythemia is a pathology whose symptoms can be found in newborn children. This disease is a response of the organism of the crumbs into transferred hypoxia, which could provoke the infant body to adjust hypoxia begins to synthesize a large number of red blood cells.

In addition to the respiratoryly due, the newborn can develop true polycythemia. This risk is especially strongly prone to twins.

Polycythemia in the newborn is developing at the first weeks of life, its first manifestations is an increase in hematocrit (up to 60%) and a significant increase in hemoglobin levels.

Neonatal polycythemia has several flow stages: the initial, phase of polyferactions and exhaustion. Briefly we describe them.

The initial stage of the disease practically does not have any clinical manifestations. It is possible to identify polycythemia in a child at this stage only by studying peripheral blood indicators: hematocrit, hemoglobin and level of red blood cells.

At the polyferaction stage, the increase in liver and spleen develops. Pleet phenomena are observed: the skin cover is acquired by the characteristic "whip-red" shade, the child is concerned with anxiety when touched to the skin. The pilor syndrome is complemented by thrombosis. In the analyzes there is a change in the number of erythrocytes, platelets and leukocyte shifts. The indicators of all blood cells can also increase, such a phenomenon is called "Pämelza".

The exposure stage is characterized by a significant loss of body weight, asthenization and exhaustion.

For a newborn, such clinical changes are extremely difficult and can provoke irreversible changes and subsequent death. Polycythemia can cause a disturbance of the development of a certain type of leukocytes, which are responsible for the body's immune system. As a result, the infant develops severe bacterial infections, resulting in death.

After reading this article, you learned more about such pathology as polycythemia. Symptoms and treatment were considered as in more detail as possible. We hope the information provided will be useful for you. Take care of yourself and be healthy!

Polycythemia is a disease characterized by an increase in the number of red blood taurus in the blood. The disease is a rare form of leukemia. This disease can be both primary due and secondary as a result of the impact of certain root causes. And the primary and secondary polycythemia are quite formidable diseases that can lead to serious consequences and chronic complications.

Polycythemia is the process of increasing the number of red blood taurus in the blood. With polycythemia, hemoglobin level (HGB), hematocrit (HCT) or red blood cells (erythrocytes) can be exceeded compared with a normal level in the study of general blood test (CBC). The level of hemoglobin is more than 16.5 g / dl (grams for decylitr) in women, and more than 18.5 g / dL in men - offers polycythemia. As for the level of hematocrit - the value exceeding the indicator 48 in women and 52 in men, testifies to polycythemia.

The production of red blood cells (erythropoes) occurs in the bone marrow and is regulated by a number of consecutive processes. One of the most important enzymes governing this process is called erythropoietin (EPO). Most of the EPO is produced by the kidneys, and the smaller part is formed in the liver. Polycythemia may be a consequence of internal problems with the production of red blood cells. This condition is called primary polycythemia. If polycythemia is due to another problem, this condition is referred to as secondary polycythemia. In the absolute majority of cases, polycythemia is secondary and due to other diseases. Primary polycythemia is a relatively rare state.

Primary causes of polycythemia

With primary polycythemia, congenital or acquired disorders with the production of red blood cells lead to polycythemia. Two basic states that belong to this category are polycythemia faith (PV or polycythemia Red Vera PRV) and primary family congenital polycythemia (PFCP).

• The polycythemia faith (PV) is associated with genetic mutation in the JAK2 gene, which is believed to increase the sensitivity of bone marrow cells to the EPO, resulting in the production of red blood cells. In this state, the level of other types of blood cells (white blood cells and platelets) is often increased.
• Primary family and congenital polycythemia (PFCP) is a state associated with a mutation in the EPOR gene, and causes an increase in the production of red blood cells in response to EPO.

True polycythemia is a disease with a purely tumor genesis. The fundamental in this disease is that stem cells are affected in the red bone marrow, and rather the ancestral cell cell of uniform blood elements (they are also called polypotent stem cells). As a result, the number of erythrocytes and other uniform elements (platelets and leukocytes) increases sharply in the body. But since the body is adapted to a certain norm of their blood content, then any exceeding limits will entail certain disorders in the body.

True polycythemia is distinguished by a rather malignant course and is difficult to treat. This is explained by the fact that it is almost impossible to influence the main cause of true polycythemia - a mutating stem cell with high mitotic activity (the ability to divide). The bright and characteristic sign of polycythemia will be a pilor syndrome. It is due to the high content of red blood cells in line. It is characterized by this syndrome of crimson-red coloring skin with a strong itching.

True polycythemia in its development passes 3 stages: the initial, deployed and terminal:

• I stage (initial, low-stream) - lasts about 5 years; It proceeds asymptomatic or with minimally pronounced clinical manifestations. Characterized by moderate hypervolemia, small erythrocytosis; The size of the spleen is normal.

• The stage II (eritremic, deployed) is divided into two substards:

  • IIA - without myeloid transformation of the spleen. Erythrocytosis, thrombocytosis, sometimes - pancitosis; According to the myelogram - hyperplasia of all sprouts of blood formation, pronounced megakaryocytosis. The duration of the deployed stage of Eritremia is 10-20 years.
  • IIIL - with the presence of myeloid metaplasia of the spleen. Expressed hypervolemia, hepato and splenomegaly; In peripheral blood - pancytosis.
• III Stage (anemic, post-credential, terminal). Characteristic anemia, thrombocytopenia, leukopenia, myeloid transformation of the liver and spleen, secondary myelofibrosis. Possible outcomes of polycythemia in other hemoblatosis.

Secondary causes of polycythemia

In contrast to the primary polycythemia, in which overproduction of red blood cells occurs as a result of increased sensitivity or reaction to EPO (often to lower than the normal level of EPO), with secondary polycythemia, the excess amount of red blood cells is formed due to the high level circulating in the blood River epoch.

The main reasons are higher than normal, the level of EPO are chronic hypoxia (insufficient level of oxygen in the blood for a long time), insufficient oxygen flow due to the abnormal structure of red blood cells, and tumors that produce an excessively high amount of EPO.

A number of common conditions that can lead to an increase in erythropoietin due to chronic hypoxia or poor oxygen supply include:

• Chronic obstructive pulmonary disease (COPD, lung emphysema, chronic bronchitis),
• Pulmonary hypertension
• Hypoventilation syndrome,
• Stagnant heart failure
• Apnea obstructive sleep,
• Insufficient blood flow to the kidneys
• Accommodation in high-mountainous areas.

2,3-BPG deficiency is a condition in which hemoglobin molecules in red blood cells have an abnormal structure. In this state, hemoglobin acquires a higher ability to attach oxygen molecules and the low ability to release oxygen in the body tissues. This leads to the production of a higher amount of red blood cells - as a reaction to the fact that the body tissues are perceived as an insufficient oxygen level. The result is a greater number of circulating erythrocytes.

Some tumors tend to identify an excessively high amount of EPO, which leads to polycythemia. Common tumors released by EPO are:

• Liver cancer (hepatocellular carcinoma),
• Kidney cancer (renal cell cancer)
• Adenoma of adrenal glands or adenocarcinoma,
• Uterine cancer.

There are also softer states that can lead to an increase in EPO secretion, such as kidney cysts and renal obstruction. The polycythemia can lead to chronic effects of carbon monoxide. Hemoglobin has a higher ability to attach carbon monoxus molecules than oxygen molecules. Therefore, when carbon monoxide molecules are attached to hemoglobin, erythrocytosis may occur as a reaction (increasing the level of erythrocytes and hemoglobin) - as compensation for the lack of oxygen delivery with existing hemoglobin molecules. Such a situation may occur with carbon dioxide with long-term smoking of cigarettes.

Polycythemia in newborns (neonatal polycithimera) often occurs when the transfer of maternal blood from the placenta or during blood transfusion. The continuous lack of oxygen transportation of the fetus (intrauterine hypoxia) due to the insufficiency of the placenta can also lead to polycythemia of newborns.

Relative polycythemia

Relative polycythemia is characterized by a condition in which the volume of red blood cells becomes elevated due to an increase in the concentration of erythrocyte blood concentrations as a result of dehydration. In such situations (vomiting, diarrhea, increased sweating), the amount of erythrocytes in the blood is normal, but due to the loss of blood-related fluid (plasma), the level of erythrocytes in the blood may seem enhanced.

Stressful polycythemia

This is a state that can be detected in men of middle-aged, hard working, with a high level of anxiety. The disease develops due to low volume of plasma, although the volume of erythrocytes may be normal. Another name for this condition is the polycythemia of risk factors.

Some of the risk factors for polycythemia:

• Chronic hypoxia;
• Long-term smoking;
• Family history and genetic predisposition;
• Accommodation in highlands;
• Long-term impact of carbon monoxide (work in mines, serving garage personnel, residents of the most polluted cities),
• Ashkenazi of Jewish origin (the frequency of polycythemia may be increased due to genetic predisposition).

Symptoms of polycythemia

The symptoms of polycythemia may vary widely. Some people with polycythemia may not be any symptoms at all. With secondary polycythemia, most symptoms are associated with the main disease responsible for polycythemia.

The symptoms of polycythemia can be vague and wear a very general character. Some important symptoms:

• Easy formation of bruises;
• Easily emerging bleeding;
• Blood clots (potentially leading to cardiac attacks, strokes, the formation of thromboms in the lungs [pulmonary embolism]);
• Pain in the joints and bones (hip pain or pain in ribs);
• Itching after taking a shower or bath;
• Fatigue;
• Stomach ache.

When to seek medical help

People with primary polycythemia need to know some potentially serious complications that they can arise. The formation of blood clots (heart attacks, strokes, blood clots in the lungs [pulmonary embolism] or legs [deep vessel thrombosis]) and uncontrolled bleeding (nasal bleeding, gastrointestinal bleeding) usually require immediate medical attention from the attending physician or emergency departments. Patients with primary polycythemia, as a rule, should take care of primary health care, consultations of therapists, family doctors, hematologists (doctors that specialize in blood diseases).

The conditions leading to secondary polycythemia can be controlled using primary links and therapists in addition to those skilled in the art. For example, people with chronic lung diseases can regularly visit their specialist (pulmonic) and individuals with chronic heart disease can regularly undergo inspection at the cardiologist.

Analyzes and tests

In most cases, polycythemia can be found randomly with a routine study of blood samples, which prescribed a doctor in connection with other medical reasons. Further research may be needed to find the causes of polycythemia.

When examining patients with polycythemia, a detailed history of the disease, a physical examination, the history of the family, social and professional history is very important. With a medical examination, special attention can be paid to the heart and surveys of the lungs. The increase in the spleen (splenomegaly) is one of the characteristic features of polycythemia, therefore a thorough examination of the abdominal cavity is carried out so as not to miss the increase in the spleen, which is of great importance.

Planned blood tests, including clinical blood test (CBC), analysis of blood sharing and its metabolic composition are the main components of laboratory tests when evaluating the causes of polycythemia. Other typical tests that will help determine the possible causes of polycythemia include chest radiography, electrocardiogram (ECG), echocardiography, hemoglobin analysis and carbon monoxide measurement.

In polycythemia, as a rule, other blood cells are also affected, for example, an abnormally high number of leukocytes (leukocytosis) and platelets (thrombocytosis) are found. Sometimes it is necessary to study the bone marrow (aspiration of bone marrow or biopsy) to explore the production of blood cells in the bone marrow. The guidelines also recommend checking the JAK2 gene mutation as a diagnostic criterion for polycythemia.

Verification of the EPO level is not mandatory, but sometimes it can provide useful information. With primary polycythemia, the level of EPO is usually low, whereas with EPO-release tumors, the level may be higher than usual. The results should be interpreted carefully, since the level of EPO can be appropriately high in response to chronic hypoxia, if it is the main cause of polycythemia.

Treatment of polycythemia

Treatment of secondary polycythemia depends on its cause. Additional oxygen can be granted to patients with chronic hypoxia. Other treatments can be aimed at treating the cause of polycythemia (for example, corresponding to the treatment of heart failure or chronic lung diseases).

The treatment of primary polycythemia plays an important role in improving the results of the disease.

Treatment at home

For persons with primary polycythemia, some simple measures at home can be taken to control potential symptoms and in order to avoid possible complications.

• It is important to maintain a sufficient water balance of the body to avoid even greater dehydration and increase blood concentration. In general, there are no restrictions in physical activity.
• If a person has increased the spleen, contact sports should be avoided to prevent damage to the spleen and its gap.
• It is best to avoid consumption of products containing iron, as this can contribute to increasing the level of red blood cells.

Treatment and therapy

The basis of the therapy during polycythemia remains bloodletting. The purpose of bloodletting is to preserve hematocrit about 45% in men and 42% in women. Initially, it may be necessary to make blood consumption every 2 - 3 days and remove from 250 to 500 milliliters of blood with each procedure. Once the target is achieved, bloodletting can be done less often to maintain the achieved level.

The usually recommended preparation for the treatment of polycythemia is hydroxyuropean (hydraulic). This drug is especially recommended for people subject to risk of thromboms. At the age of patients over 70, having an increased amount of platelets at the same time (thrombocytosis) and cardiovascular diseases, the use of hydroxymes makes more results more favorable. Hydroxyimoevin is also recommended for patients who do not tolerate bleeding.

Aspirin is also used in the treatment of polycythemia to reduce the risk of blood coagulation (thrombus formation). Its use, as a rule, should be avoided by those people who had any bleeding in history. Aspirin is usually used in combination with bleeding.

Following actions

At the beginning of treatment, blood consumption is recommended thorough and regular control, until the acceptable level of hematocrit. After this bloodletting can be carried out as needed to maintain the appropriate level of hematocrit, based on the reaction of each patient to this therapy.

Some of the complications of primary polycythemia, as indicated below, often require more close observation and monitoring. These complications include:

• The formation of blood clots (thrombosis), which cause heart attacks, strokes, thrombus in the legs and lungs, or thrombus in the arteries. These events are considered the main causes of death from polycythemia.
• Heavy blood loss or bleeding.
• Transformation of polycythemia in blood cancer (for example, leukemia, myelofibrosis).

Prevention

Many of the reasons for secondary polycythemia cannot be prevented. Nevertheless, there are some potential preventive measures:

• To give up smoking;
• Avoiding the long-term effects of carbon monoxide;
• Proper management of diseases such as chronic lung diseases, heart disease or obstructive sleep apnea.

Primary polycythemia due to the gene mutation, as a rule, is not prevented.

Forecast

Prospects for the development of primary polycythemia without treatment are usually unfavorable, with a lifetime of about 2 years. Nevertheless, the forecasts are significantly improved and increased by more than 15 years expected lifespan, when using bleeding, the forecasts of secondary polycythemia are largely dependent on the underlying disease.

True polycythemia (eritrium, vacuise disease or primary polycythemia) is a progressive malignant disease relating to leukemia, which is associated with hyperplasia of bone marrow cell elements (myeloproliferation). The pathological process affects the erythrobist sprout mainly, therefore the excess amount of red blood cells are detected in the blood. It is also observed an increase in the number of neutrophil leukocytes and platelets.

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The increased amount of erythrocytes increases blood viscosity, increases its mass, causes a slowdown in blood flow in vessels and blood cloves. As a result, patients develop a violation of blood supply and hypoxia.

General

True polycythemia was first described in 1892 by French and Vaquez (Vaquez). The Vaces suggested that hepatosplegilee and erythrocytosis, revealed in his patient, arose as a result of the reinforced proliferation of hematopoietic cells, and allocated eritrium into a separate nosological form.

In 1903, V. Osler to describe patients with splenomegaly (increasing spleen) and expressed erythrocytosis used the term "vacawa disease" and gave a detailed description of the disease.

Turk (W. TURK) In 1902-1904, it suggested that in this disease, the violation of blood formation is hyperplastic, and called Eritrea's disease by analogy with leukemia.

The clonal neoplastic nature of myeloproliferation, which is observed in polycythemia, proved in 1980 by Phials (P. J. Fialkov). It discovered in red blood cells, granulocytes and platelets one type of glucose-6-phosphate dehydrogenase enzyme. In addition, both types of this enzyme were detected in the lymphocytes of two heterozygous patients in this enzyme. Thanks to the studies of Phialkov, it became clear that the target of the neoplastic process is the predecessor cell of myelopoies.

In 1980, a number of researchers managed to separate a neoplastic clone from normal cells. It was experimentally proved that with polycythemia, the population of erythroid comed by predecessors, which have pathologically high sensitivity even to a small amount of erythropoietin (kidney hormone) are formed. According to the assumption of scientists, this contributes to the increased formation of erythrocytes in true polycythemia.

In 1981, L. D. Sidorova and co-authors conducted research that allowed to detect high-quality and quantitative changes in the thrombocytene ring of hemostasis, playing a major role in the development of hemorrhagic and thrombotic complications in polycythemia.

True polycythemia is revealed mainly in the elderly, but can be observed in young people, and in children. In people of young age, the disease occurs in a more severe form. The average age of patients varies from 50 to 70 years. The average age for the first time the diseased gradually increases (in 1912, amounted to 44 years, and in 1964 - 60 years). The number of patients under 40 years of age is about 5%, and eritremia in children and patients under 20 years of age is revealed in 0.1% of all cases of the disease.

Eritremia is somewhat less common in women than in men (1: 1.2-1.5).

This is the most common disease in the group of chronic myeloproliferative diseases. It is quite rare - according to various data from 5 to 29 cases per 100,000 population.

There are isolated data on the influence of racial factors (above average among the Jews and below average among representatives of the Negroid race), but at the moment this assumption is not confirmed.

Forms

True polycythemia is divided into:

• Primary (not a consequence of other diseases).
• Secondary. It may be triggered by a chronic pulmonary disease, hydronephrosis, the presence of tumors (fibroma of the uterus, etc.), the presence of abnormal hemoglobins, etc. factors associated with tissue hypoxia.

The absolute increase in the mass of erythrocytes is observed in all patients, but only 2/3 also increases the number of leukocytes and platelets.

Causes of development

The reasons that cause true polycythemia are finally established. Currently, there is no uniform theory that would explain the occurrence of hemoblastosis (blood tumors), to which this disease belongs.

Based on epidemiological observations, the theory of eritremia with the transformation of stem cells was put forward, which occurs under the influence of gene mutations.

It has been established that most patients have a mutation that is synthesized in the liver of the enzyme-kinase-tyrosine kinase enzyme, which participates in the transcription of those or other genes by phosphorylation of many tyrosins in the cytoplasmic part of the receptors.

Most often occurred in 2005, the mutation in 14 exon JAK2V617F (detected in 96% of all cases of the disease). In 2% of cases, the mutation affects 12 JAK2 genes.

Patients with true polycythemia are also detected:

• In some cases, mutations in the MPL thrombopoietin receptor gene. These mutations have a secondary origin and are not strictly specific for this disease. It is detected in the elderly people (mainly in women) with a low level of hemoglobin and platelets.
• Loss of the function of the LNK genet SH2B3 protein, which reduces the activity of the JAK2 gene.

For elderly patients with high allelic loading, JAK2V617F is characterized by an elevated hemoglobin, leukocytosis and thrombocytopenia.

When mutations, the JAK2 gene in 12 eritrium exon is accompanied by a subnormal level in the serum of the hormone erythropoietin. Patients with this mutation are characterized by younger age.
With true polycythemia, TET2, IDH, ASXL1, DNMT3A, etc., and other mutations are also detected, but their pathogenetic significance has not yet been studied.

Differences in the survival of patients with different types of mutation was not detected.

As a result of molecular genetic disorders, the JAK-STAT signal path is activated, which is manifested by proliferation (cell production) of myeloid sprout. At the same time, proliferation increases and an increase in the number of erythrocytes in peripheral blood (it is also possible to increase the number of leukocytes and platelets).

The identified mutations are inherited by autosomal-recessive type.

There is also a hypothesis, according to which the cause of Eritremia may be viruses (15 species of such viruses have been identified), which, in the presence of predisposing factors and weakening immunity penetrate into immature bone marrow cells or lymph nodes. The cells affected by the virus begin instead of maturation to be actively divided, thus running the pathological process.

To provoking disease factors include:

• x-ray irradiation, ionizing radiation;
• paints, varnishes and other toxic substances penetrating into the human body;
• long-term use in therapeutic purposes of some drugs (salt of gold with rheumatoid arthritis, etc.);
• viral and intestinal infection, tuberculosis;
• surgical interventions;
• stressful situations.

Secondary eritremium develops under the influence of conducive factors when:

• high congenital affinity of hemoglobin to oxygen;
• low level of 2,3-diphosphoglycerat;
• autonomous erythropoietin products;
• arterial hypoxemia of physiological and pathological nature ("blue" defects of the heart, smoking, adaptation to the conditions of the highlands and chronic diseases of the lungs);
• kidney diseases (cystic lesions, hydronephrosis, stenosis of renal arteries and diffuse diseases of the renal parenchyma);
• the presence of tumors (the effect of bronchi carcinoma, cerebellum hemangiobrestoma, uterine fibromes);
• endocrine diseases associated with adrenal tumors;
• liver diseases (cirrhosis, hepatitis, hepatoma, badda-kiaari syndrome);
• tuberculosis.

Pathogenesis

The pathogenesis of true polycythemia is associated with a violation of the blood formation process (hematopoede) at the level of the predecessor cell. Hematopois acquires an unlimited proliferation of the predecessor cells for the tumor, the descendants of which form a specialized phenotype in all sprouts of blood formation.

True polycythemia is characterized by the formation of erythroid colonies in the absence of exogenous erythropoietin (the occurrence of endogenous erythropoietin-independent colonies is a sign that allows Eritrees from secondary red blood cell).

The formation of erythroid colonies indicates a violation of the process of implementing regulatory signals, which myeloid cell receives from the external environment.

The basis of the pathogenesis of true polycythemia is defects of genes encoding proteins that are responsible for maintaining myelopoes in the norm.

Reducing the oxygen concentration causes the reaction of interstitial kidney cells, synthesizing erythropoietin. The process occurring in interstitial cells relates to the work of many genes. The main regulation of this process is carried out using factor-1 (HIF-1), which consists of two subunits (HIF-1ALPHA and HIF-1BETA) with a heterodimer protein.

If the concentration of oxygen in the blood is under the boundaries of the norm, the remains of the proline (heterocyclic amino acid of the freely existing HIF-1 molecule) are hydroxylized under the influence of the regulatory enzyme PHD2 (molecular oxygen sensor). Due to the hydroxylation, the HIF-1 subunit acquires the ability to bind to VHL protein that ensures the prevention of tumors.

The VHL protein forms a complex with a number of E3-Revivitin-ligase proteins, which, after the formation of covalent bonds with other proteins, are sent to proteasury and are destroyed there.

In hypoxia, the hydroxylation of the HIF-1 molecule does not occur, the subunit of this protein is combined and form a heterodimeric HIF-1 protein heading from the cytoplasm into the kernel. The protein in the core binds to the industrial areas of genes with special DNA sequences (conversion of genes in protein or RNA is induced by hypoxia). As a result of these transformations by interstitial kidney cells, erythropoietin is released into the bloodstream.

The predecessors of myelopoese embedded in them the genetic program is carried out as a result of the stimulating effect of cytokines (these small peptide control (signaling) molecules are associated with the corresponding receptors on the surface of the presenters cells).

When binding erythropoietin with the ERITROTOETET EPO-R receptor, there is a dimerization of this receptor, which activates the EPO-R JAK2-kinase associated with intracellular domains.

JAK2 kinase is responsible for the transmission of the signal from erythropoietin, thrombopoietin and G-CSF (is a colony positive factor of granulocytes).

Due to the activation of JAK2-kinase, phosphoria of a number of cytoplasmic target proteins occurs, which include the adapter proteins of the STAT family.

Eritremia was revealed in 30% of patients with constitutive activation of the STAT3 gene.

Also, in order, in some cases, a reduced level of expression of the MPL thrombopoietin receptor is revealed, which is compensatory. The decrease in the expression of MPL is secondary and is caused by a genetic defect responsible for the development of true polycythemia.

Reducing the degradation and improving the level of the HIF-1 factor is caused by the VHL gene defects (as well as the representatives of the population of Chuvashia characteristic of the homozygous mutation of 598C\u003e T of this gene).

True polycythemia can be caused by anomalies of 9 chromosomes, but most often the deletion of the long shoulder 20 chromosome is observed.

In 2005, they revealed a point mutation 14 of the exon of the JAK2-kinase gene (JAK2V617F mutation), which causes replacing the amino acid valine phenylalanin in the JAK2 protein pseudocinase domain in the 617 position.

JAK2V617F mutation in hematopois precursor cells during eritrium is represented in homozygous form (mitotic recombination and duplication of a mutant allele affect the formation of a homozygous form).

With JAK2V617F and STAT5 activity, an increase in the level of the active forms of oxygen is occurring, as a result of which the transition of the cell cycle from the G1 phase in S is observed. The STAT5 adapter protein and the active oxygen forms transmit a regulatory signal from JAK2V617F Cycline D2 and P27KIP genes, which causes an accelerated transition of the cell cycle from Phases G1 in S. As a result, the proliferation of erythroid cells is enhanced, which carry the mutant shape of the JAK2 gene.

In JAK2V617F-positive patients, this mutation is detected in myeloid cells, in B- and T-lymphocytes and cells of natural killers, which proves the proliferative advantage of defective cells compared with the norm.

True polycythemium in most cases is characterized by a sufficiently low ratio of mutant and normal allele in mature myeloid cells and early predecessors. In the presence of clonal domination, patients have a heavier clinical picture in comparison with patients without this defect.

Symptoms

The symptoms of true polycythemia are associated with excessive erythrocyte production that increase blood viscosity. In most patients, platelet levels are also increased, which cause vascular thrombosis.

The disease develops very slowly and at the initial stage is asymptomatic.
At the later stages, true polycythemia manifests itself:

• pletic syndrome, which is associated with enhanced blood circulating;
• myeloproliferative syndrome, which occurs with the increased formation of erythrocytes, platelets and leukocytes.

Pletic syndrome is accompanied by:

• Head pains.
• Feeling gravity in the head;
• Dizziness.
• Attachments of the grain, compressing the pain behind the sternum, which occurs during exercise.
• Erythrocyanosis (redness of the skin to the cherry shade and a blue tint of the tongue and lips).
• Redness of the eye, which arises as a result of the extension of vessels in them.
• Feeling gravity in the upper abdomen (left), which arises as a result of an increase in the spleen.
• Skin itch, which is observed in 40% of patients (specific feature of the disease). Strengthens after water procedures and arises as a result of irritation by products of the decay of erythrocytes of nerve endings.
• An increase in blood pressure, which is well reduced during bloodletting and slightly decreases with standard treatment.
• Erythrolealgia (sharp burning pains in the tips of the fingers, which decrease as a result of the reception of the blood thinning drugs, or painful edema and redness of the foot or lower third of the leg).

Myeloproliferative syndrome manifests itself:

• soreness in flat bones and pain in the joints;
• feeling of gravity in the right upper part of the abdomen as a result of the increase in the liver;
• total weakness and increased fatigue;
• increase body temperature.

There is also an expansion of veins, especially noticeable in the neck, the symptom of Cooperman (change in the color of the soft sky with normal color of the solid sky), duodenal ulcer and in some cases the stomach, bleeding gum and esophagus, an increase in uric acid level. It is possible to develop heart failure and cardiosclerosis.

Stages of the disease

For true polycythemia, three stages of development are characteristic:

• Initial, stage I, which lasts about 5 years (possible and longer periods). It is characterized by moderate manifestations of the pilor syndrome, the size of the spleen does not exceed the norm. Common blood test identifies a moderate increase in the number of erythrocytes, an enhanced formation of erythrocytes is observed in the bone marrow (it is also possible to increase the number of all blood cells, with the exception of lymphocytes). At this stage, there is practically no complications.
• The second stage, which can be polycytemetic (II A) and polycytemic with myeloid metaplasia of the spleen (II b). Form II A, which lasts from 5 to 15 years, is accompanied by pronounced plaque syndrome, an increase in liver and spleen, the presence of thrombosis, bleeding. Tumor growth in the spleen is not detected. Iron deficiency occurs due to frequent bleeding. Common blood test identifies the increase in the number of erythrocytes, platelets and leukocytes. In the bone marrow, scar changes are observed. For Form II B, a progressive increase in liver and spleen is characterized, the presence of tumor growth, thrombosis, general exhaustion, and the presence of bleeding. The overall blood test makes it possible to detect an increase in the number of all blood cells, with the exception of lymphocytes. Erythrocytes acquire different sizes and shapes, immature blood cells appear. Scar changes in the bone marrow are gradually growing.
• The anemic, III stage, which develops 15-20 years after the start of the disease and is accompanied by a pronounced increase in liver and spleen, extensive scar changes in the bone marrow, circulatory disorders, a decrease in the number of erythrocytes, platelets and leukocytes. Transformation is possible in acute or chronic leukemia.

Diagnostics

Eritremia is diagnosed based on:

• Analysis of complaints, anamnesis of the disease and family history, in the process of which the doctor clarifies when symptoms of the disease appeared, which chronic diseases are in the patient, was a contact with toxic substances, etc.
• Data of physical inspection, in which attention is drawn to the color of the skin. In the process of palpation and with the help of percussion (clutch), the size of the liver and spleen is determined, the pulse and blood pressure are also measured (it can be elevated).
• The analysis of the blood at which the number of erythrocytes is determined (the rate of 4.0-5.5x109g / l), leukocytes (may be normal, increased or reduced), platelets (at the initial stage does not deviate from the norm, then there is an increase in the level, and then a decrease ), hemoglobin level, color indicator (normally revealed norm - 0.86-1.05). ESO (erythrocyte sedimentation rate) in most cases reduced.
• Urine Analysis, which allows you to identify related diseases or renal bleeding.
• Biochemical analysis of blood, allowing to identify the elevated level of uric acid characteristic of many cases. To identify the concomitant disease, damage to organs is also determined by the level of cholesterol, glucose, etc.
• Data Studies of bone marrow, which is carried out using puncture in the field of sternum and allows you to identify an increased formation of erythrocytes, platelets and leukocytes, as well as education in the bone marrow of scar tissue.
• Tpardobiopia data, which most fully reflect the condition of the bone marrow. For the study using a special instrument, the bone marrow bar is taken from the wing of the ileal bone together with the bone and the periosteum.

Coagulogram, iron exchange studies are also carried out and the level of erythropoietin in serum is determined.

Since chronic eritremium is accompanied by an increase in liver and spleen, an ultrasound of internal organs is carried out. With the help of ultrasound, the presence of hemorrhages is also detected.

To evaluate the prevalence of the tumor process, CCT (spiral computed tomography) and MRI (magnetic resonance tomography).

To identify genetic anomalies, a molecular genetic study of peripheral blood is carried out.

Treatment

The goal of treatment with true polycythemia is:

• preventing and therapy of thrombohemorrhagic complications;
• elimination of symptoms of the disease;
• reducing the risk of developing complications and the development of acute leukemia.

Eritremia is treated with:

• Bloodstocks, in which 200-400 ml of blood in young people and 100 ml of blood is removed to reduce blood viscosity of the blood and 100 ml of blood in concomitant heart diseases or in older people. The course is 3 procedures that are conducted with an interval of 2-3 days. Before the procedure, the patient takes preparations that reduce blood clotting. Bloodstocks are not conducted in the presence of recent thrombosis.
• Hardware treatment methods (erythrocytaferesis), with which the excess of erythrocytes and platelets are removed. The procedure is carried out at intervals of 5-7 days.
• Chemotherapy, which is used on the II of the stage, in the presence of an increase in the number of all blood cells, poor tolerance of blood consumption or the presence of complications from the internal organs or vessels. Chemotherapy is carried out according to a special scheme.
• Symptomatic therapy, including hypotensive drugs with increased blood pressure (ACE inhibitors are usually prescribed), antihistamine drugs to reduce skin serium, disagreganites that reduce blood clotting, hemostatic drugs during bleeding.

Anticoagulants are used to prevent thrombosis (acetylsalicylic acid is usually assigned to 40-325 mg / day).

Eritremium food must comply with the requirements of the healing table according to Pevznera No. 6 (the number of protein products is reduced, fruits and vegetables are excluded and products with dyes content).

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