Polycythemia in newborns treatment. Transient (transitional) states of newborns during the adaptation period

Date: 04.07.2020

The lecture was given by: MD, prof. Pyasetskaya N.M., dept. Neonatology on the basis of the Ukrainian Children's Specialized Hospital of the Ministry of Health of Ukraine "OKHMATDET".

Polycythemia of the newborn

Polycythemia - This is a malignant increase in the number of cells of blood germs: erythroid to a greater extent, platelet and neutrophilic to a lesser extent.

ICD-10 code: R61, R61.1

Clinical diagnosis:

Neonatal polycythemia (erythrocytosis, primary polycythemia, true) is diagnosed when:

Ht veins. (Venous hematocrit)\u003e 70% or venous Hb\u003e 220 g / l.

An example of a diagnosis:Primary polycythemia with severe erythrocytosis, thrombocytosis and leukocytosis, grade II. (erythremic stage). Hepatosplenomegaly. Vascular thrombosis.

The incidence is:

2-5% - in healthy full-term newborns,

7-15% - in premature babies.

Polycythemia problem:

reduced transport function of erythrocytes;
oxygen supply to tissues is impaired (Ht veins\u003e 65%).

The causes of polycythemia:

1) Intrauterine hypoxia (increased erythropoiesis):

gestosis of pregnant women;
severe heart disease of the mother;
placental insufficiency of an infant with intrauterine hypotrophy;
postmaturity (additional fluid loss);

2) Lack of oxygen delivery (secondary polycythemia of the newborn):

impaired ventilation (lung disease);
congenital blue heart defects;
congenital methemoglobinemia;

3) Risk group for the development of neonatal polycythemia in newborns:

Diabetes mellitus in the mother;
Late clamping of the umbilical cord (\u003e 60 sec);
Feto-fetal or maternal-fetal transfusion;
Congenital hypothyroidism, thyrotoxicosis;
Down syndrome;
Wiedemann-Beckwith syndrome;

Classification of polycythemia in newborns:

1) Neonatal polycythemia:

2) Primary polycythemia:

Polycythemia vera;
Erythrocytosis (benign familial polycythemia of the newborn);

3) Secondary polycythemia - the result of insufficient oxygen delivery (promotes the synthesis of erythropoietin, which accelerates erythropoiesis and increases the number of red blood cells), or a failure in the hormone production system.

A. Oxygen deficiency:

Physiological: during intrauterine development; low oxygen content in the inhaled air (high altitude).
Pathological: impaired ventilation (lung disease, obesity); arteriovenous fistulas in the lungs; congenital heart disease with an intracardiac shunt from left to right (tetrad of Fallot, Eisenmenger complex); hemoglobinopathies: (methemoglobin (congenital or acquired); carboxyhemoglobin; sulfhemoglobin; hemoglobinopathies with a high affinity of hemoglobin for oxygen; lack of 2,3-diphosphoglycerate mutase in erythrocytes.

B. Enhanced erythropoiesis:

Endogenous causes:

a) on the part of the kidneys: Wilms tumor, hypernephroma, renal ischemia, vascular diseases of the kidneys, benign neoplasms of the kidneys (cysts, hydronephrosis);

b) from the adrenal glands: pheochromocytoma, Cushing's syndrome, congenital adrenal hyperplasia with primary aldosteronism;

c) from the liver: hepatoma, focal nodular hyperplasia;

d) from the side of the cerebellum: hemangioblastoma, hemangioma, meningioma, hepatocellular carcinoma, liver hemangioma;

e) from the side of the uterus: leiomyoma, leiomyosarcoma.

Exogenous causes:

a) the use of testosterone and related steroids;

b) the introduction of growth hormone.

4) False (relative, pseudocythemia).

Gaisbeck's syndrome also refers to false polycythemia, since it is characterized by an increase in the level of red blood cells in a general blood test and an increase in blood pressure, which, in combination, gives similar clinical manifestations, as well as polycythemia, but hepatosplenomegaly and the appearance of immature forms of leukocytes are not observed.

Stages of neonatal polycythemia:

I Art. (initial) - the clinical picture is erased, the disease is sluggish. The first stage can last up to 5 years. The disease can be suspected only with a laboratory blood test, in which moderate erythrocytosis is observed. Objective data are also not very informative. The spleen and liver are slightly enlarged, but this is not a pathognomonic sign of this disease. Complications from internal organs or blood vessels are extremely rare.

II Art. (proliferation) - the clinic is characterized by the height of the disease. There is a pletora, hepatosplenomegaly, exhaustion of the body, manifestation of thrombosis, convulsions, tremor, dyspnea. In the general analysis of blood - erythrocytosis, thrombocytosis, neutrophillosis with a shift to the left, or panmyelosis (an increase in the amount of all blood elements). In the blood serum, the content of uric acid rises (norm \u003d up to 12 years - 119-327 μmol / l), which is synthesized in the liver and excreted by the kidneys. It circulates in blood plasma in the form of sodium salts.

III (emaciation, anemic) - clinical signs in the form of pletora, hepatosplenomegaly, general weakness, significant weight loss. At this stage, the disease becomes chronic and myelosclerosis may occur.

Syndromes that are accompanied by an increased level of Ht veins.

Blood hyperviscosity (not synonymous with polycythemia) is the result of increased levels of fibrinogen, IgM, osmolarity and blood lipids. Addiction with polycythemia becomes exponential when Htwen exceeds 65%.
Hemoconcentration (relative polycythemia) is an increased level of hemoglobin and hematocrit due to a decrease in plasma volume due to acute dehydration of the body (exsicosis).

General Clinic for Polycythemia:

Pletora (with primary polycethemia) is a general plethora of the body. There is a reddening of the face (becomes purple), a strong, high pulse, "beating in the temples", dizziness.
Insufficient filling of capillaries (acrocyanosis).
Dyspnea, tachypnea.
Depression, drowsiness.
Weakness in sucking.
Persistent tremor, muscle hypotonia.
Convulsions.
Bloating.

Complications (clinical conditions associated with polycythemia and hemoconcentration syndrome (thickening) of the blood):

Pulmonary hypertension with the development of PFC syndrome (persistent fetal circulation).
Increased systemic blood pressure.
Venous congestion in the lungs.
Increased tension on the myocardium.
Hypoxemia.
Metabolic disorders (hyperbilirubinemia, hypocalcemia, hypomagnesemia).
Increased glucose utilization (hypoglycemia)
Hepatomegaly.
Intracranial hemorrhage, convulsions, apnea.
Renal vein thrombosis, acute renal failure (acute renal failure), oliguria.
Ulcerative necrotizing enterocolitis.
Decreased blood circulation in the gastrointestinal tract, kidneys, brain, myocardium.

Diagnostics.

Laboratory data:

Ht veins
general blood analysis

It should be remembered that 4-6 hours (sometimes earlier) after birth, hemoconcentration necessarily occurs (rise in hematocrit, hemoglobin, leukocytes) due to certain physiological mechanisms.

Additional examinations:

platelets (thrombocytopenia),
blood gases
blood sugar (hypoglycemia),
bilirubin (hyperbilirubinemia),
urea,
electrolytes,
x-ray of the lungs (with RDS).

If necessary (determination of blood hyperviscosity), determine fibrinogen, IgM, blood lipids, calculate the osmolarity of the blood.

Differential diagnosis of true neonatal polycythemia, true secondary polycythemia due to hypoxia and pseudo-polycythemia (relative).

True neonatal polycythemia:

There is granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of erythrocytes is increased;
The regulator of erythropoiesis (erythropoietin) is normal or decreased;

True secondary polycythemia due to hypoxia:

The mass of erythrocytes is increased;
Plasma volume unchanged or reduced;
The regulator of erythropoiesis (erythropoietin) is increased;
Decreased or normal arterial oxygen saturation.

Pseudo-polycythemia:

No granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of red blood cells is unchanged;
Plasma volume is reduced;
The erythropoiesis regulator (erythropoietin) is normal;
Normal arterial oxygen saturation.

Treatment of polycythemia.

1) General activities:

Ht vein level control:

a) with Ht veins 60-70% + no clinical signs \u003d control after 4 hours

b) with Ht veins\u003e 65% + clinical signs \u003d normovolemic hemodilution or partial exchange transfusion (exfusion).

Repeated control of Ht veins: 1, 4, 24 hours after hemodilution or partial exchange transfusion

Normovolemic hemodilution:

Purpose: to reduce the level of Ht veins to 50-55% by diluting the blood (more often this method is used in the presence of dehydration).

Partial exchange transfusion:

Purpose: to reduce blood viscosity (reduce the level of Ht veins to 50-55%) by successive replacement (exfusion) of the child's blood with infusion solutions of equal volume (10-15 ml each) (see the formula for calculating the desired volume)

Formula for calculating the required volume (ml) of exfusion - infusion or hemodilution:

V (ml) \u003d BCC child (ml / kg) * (Ht child - Ht desired) / Ht child, where

V (ml) - volume of partial exchange transfusion (infusion)

Ht desired ≈ 55%

BCC of a full-term baby - 85-90 ml / kg

BCC of a premature baby - 95-100 ml / kg

Child's HT - 71%;

Ht desired - 55%;

BCC of a child - 100 ml / kg;

Child's body weight - 3 kg

V (ml) \u003d 100 x 3 x (71% - 55%) 300 ml x 16% / 71% \u003d 67.6 ml. or 17 ml. x 4 times *

* Note: Do not use the pendulum technique. This technique increases the risk of developing necrotizing enterocolitis. It is necessary to carry out simultaneously in equal volumes of exfusion - transfusion using different vessels.

Solutions that can be used for hemodilution and partial exchange transfusion:

physiological solution (0.85% sodium chloride solution);
ringer's solution or Ringer's lactate;
colloidal solutions based on hydroxyethyl starch (HES) - 6%, 10% Refortan solution (the indication for the use of this solution is hemodilution, correction of hemodynamic disturbances, improvement of rheological properties of blood, and others). There is little experience in neonatology.

Human plasma (FFP) should not be used.

Forecast.

If it is impossible to carry out exchange transfusion of plasma, neurological disorders may occur: general developmental delay, dyslexia (speech disorders), impaired development of various types of movement, but exchange transfusion does not exclude the possibility of neurological disorders.

With latent (asymptomatic) polycythemia, the risk of neurological disorders increases.

Regional maternity hospital

"APPROVED"

Chief physician of the ORD

M. V. Mozgot

"________" _____________________ 2007

Yaroslavl - 2009

List of abbreviations

IUGR - intrauterine growth retardation

BCC - the volume of circulating blood

ChIG - partial isovolemic hemodilution

YANEK - ulcerative necrotizing enterocolitis

Ht - hematocrit

Hb - hemoglobin

Polycythemia diagnosed in newborns with a venous hematocrit of 0.65 and above, and Hb up to 220 g / l and above. The upper limit of the normal value of peripheral venous Ht is 65%. The hematocrit in a newborn reaches a maximum 6-12 hours after delivery, decreases by the end of the first day of life, reaching values \u200b\u200bin the umbilical cord blood.

Pathophysiology

Symptoms of polycythemia in newborns are caused by local manifestations of increased blood viscosity: tissue hypoxia, acidosis, hypoglycemia, the formation of microthrombi in the vessels of the microvasculature.

Risk factors for developing polycythemia

An increase in fetal erythropoiesis is secondary due to intrauterine hypoxia:

placental insufficiency as a result of preeclampsia, vasorenal pathology, repeated episodes of partial placental abruption, congenital blue-type heart defects, post-term pregnancy, mother's smoking. Most of these conditions are associated with the development of IUGR;

endocrine disorders are associated with increased oxygen metabolism in fetal tissues. Includes congenital thyrotoxicosis, Beckwith-Wiedemann syndrome, diabetic fetopathy with inadequate glucose control;

genetic disorders (trisomies 13, 18 and 21).

Hypertransfusion:

delayed clamping of the umbilical cord. A delay in clamping the umbilical cord for more than 3 minutes after delivery leads to an increase in BCC by 30%;

delaying cord clamping and exposure to uterotonic drugs lead to an increase in blood outflow to the fetus (in particular, oxytocin);

force of gravity. Depends on the position of the newborn in height relative to the mother's body before clamping the umbilical cord (if below the placenta level by more than 10 cm);

fetal-fetal transfusion syndrome (about 10% of monozygous twins);

home birth;

intranatal asphyxia leads to a redistribution of blood from the placenta to the fetus.

Clinical manifestations

Discoloration of the skin:

crimson, bright red skin color

may be normal or pale.

From the side of the central nervous system:

changes in consciousness, including lethargy,

decreased physical activity,

increased excitability (jitteriness),

difficulty sucking

convulsions.

Respiratory and cardiovascular disorders:

respiratory distress syndrome,

tachycardia,

muted tones,

congestive heart failure with low cardiac output and cardiomegaly

primary pulmonary hypertension may occur.

Gastrointestinal tract:

feeding problems,

bloating

ulcerative necrotizing enterocolitis (often not associated with polycythemia itself and occurs when colloids are used as a replacement solution during partial hemodilution, which cannot be said about crystalloids).

Genitourinary system:

acute renal failure

priapism in boys - pathological erection due to sludge of red blood cells.

Metabolic disorders:

hypoglycemia,

hypocalcemia,

hypomagnesemia.

Hematological disorders:

hyperbilirubinemia,

thrombocytopenia,

hypercoagulability with the development of disseminated intravascular coagulation,

reticulocytosis (only with increased erythropoiesis).

Polycythemia is an increase in red blood cells in the blood volume. Types of pathology:

primary values;
secondary

The secondary and primary form of the disease is difficult. The consequences for the patient's health are serious. Causes of a different type of disease:

bone marrow tumor;
the production of red blood cells is increased

The main cause of secondary damage in this disease is hypoxia. The compensatory reaction is a secondary type of polycythemia.

Polycythemia true type

In the development of polycythemia of the true type, a tumor disease plays a role. The principles of defeat in this disease:

stem cell damage;
an increased number of red blood cells;
enlargement of shaped elements

The result is severe violations. The true type of the disease is of malignant origin. Therapy is difficult to select. The reason is the lack of impact on the stem cell.

This cell is capable of division. Plethoric syndrome is a symptom of this disease. A large erythrocyte content in the blood is plethoric syndrome.

External symptoms of the syndrome:

skin coloration;
the presence of intense itching

Stage diseases are determined. The peak stage of the main features is stage one. At this stage, the main symptoms are as follows:

bone marrow phenomena;
areas of hematopoiesis are changed

When pathology is detected, the peak stage is determined. Blood tests include diagnostic methods. The stage of clinical symptoms includes:

brace syndrome;
the presence of itching of the skin;
enlarged spleen

The stage of anemia is as follows. Bone marrow hyperplasia occurs at this stage. There are severe complications. The following processes lead to the formation of blood clots:

increased erythrocyte;
platelet increase

Thrombotic lesions are formed in this situation. An increase in blood pressure leads to an increase in blood pressure. The following consequences are also possible:

intracranial hemorrhage;
hemorrhagic stroke

etiology of polycythemia

An increase in erythrocytes in the bloodstream are signs of this disease. There are types of disease:

relative view;
absolute view

An increase in the number of erythrocytes is an absolute disease. Kind of absolute variety:

true type polycythemia;
polycythemia of the hypoxic type;
kidney damage;
increased production of red blood cells

Polycythemia vera signs:

the formation of tumor cells;
the phenomenon of oxygen starvation;
increased erythropoietin formation

Signs of the relative type of disease:

an increase in the volume of red blood cells;
decrease in plasma volume;
change of shaped elements

Diseases leading to relative polycythemia:

infectious diseases;
dysentery;
salmonellosis;

Signs of these states:

vomit;
increase in water supply

Reasons for the relative type of pathology:

burns;
heat;
sweating;
tumor;
hypoxia

The mechanism of development of pathology is a sign of mutations. There are the following disorders in true type polycythemia:

increased content of erythrocytes;
increased hematopoietic system

Due to oxygen starvation, secondary type polycythemia is observed. The phenomenon of hypoxia concerns the renal system.

Processes under the influence of erythropoietin:

various features of stem cells;
erythrocyte cell formation

Bone marrow forms erythrocytes.

signs of polycythemia

The main symptom of polycythemia is pletora syndrome. Diagnosis of the patient's conditions in this case concerns an objective examination. Signs of this syndrome:

dizziness symptoms;
headache;
itchy skin;
synthesis of histamine;

Itching worsens on contact with water:

washing in the bath;
washing in the shower;
washing;
erythromelalgia of the hands;
bluish skin;
pain

The release of histamine is the cause of itchy skin. The skin is cherry-colored. The cardiovascular system is changing. Signs of damage to the heart system:

hypertension;
thrombus development;
increased systole

The secondary syndrome of the disease is characterized by an increase in internal organs. The reason is the function of the spleen. Red cells are destroyed.

The consequences of this process are:

splenic hyperplasia;
excess of red cells

Signs with an enlarged spleen:

fatigue;
asthenia;
pain of the left hypochondrium

An increase in blood viscosity contributes to the following symptoms:

thrombotic lesion;
stroke foci;
heart attack;
pulmonary thromboembolism phenomenon

Signs of the underlying pathology are:

blue skin;
chronic pathologies of the pulmonary system;
oxygen starvation

Signs are also revealed:

impairment of renal function;
tumor

The etiology of the secondary type is as follows:

the phenomenon of diarrhea;
the phenomenon of vomiting;
increased erythrocyte count

polycythemia in newborns

Polycythemia of newborns is the body's response to hypoxia. Hypoxia is a consequence of placental pathology. Oxygen starvation can result from:

heart defects;
lung pathology

Twins may develop true type polycythemia. The first week of birth is at risk. Signs:

increased hematocrit;
increase in hemoglobin

Stage polycythemia:

emaciation stage;
proliferation stage;
initial stage

Diagnostics of the first stage of the disease:

examination of the blood picture;
hemoglobin study;
study of red cells

With the development of proliferation, internal organs increase. The following symptoms appear:

red skin;
having anxiety;
the presence of blood clots;
change in blood picture;
development of panmyelosis

Signs of the stage of exhaustion:

spleen enlargement;
liver enlargement;
weight loss;
exhaustion

Death can be the outcome. Sclerosis develops with the true type of polycythemia. The production of erythrocytes is also impaired. The consequence is a bacterial infectious focus.

polycythemia - Treatment

The main reason is being identified. The search for the etiology of the disease is characteristic of a secondary disease. They have an effect on tumor cells in the true type of disease. The formation of shaped elements is stopped.

The true type of polycythemia is difficult to cure. Treatment is selected taking into account age characteristics. Therapy is an indicator of age characteristics. Drug therapy is possible in the age group from seventy years.

Treatment of the tumor process is as follows:

hydroxyurea preparation;
hydrea remedy;

A bloodletting procedure is also used. This method is effective in the true type of polycythemia. The purpose of this method is to reduce the hematocrit.

A decrease in blood volume is carried out with cardiac pathologies. Diagnostics is applied before this procedure. Diagnostics include:

determination of hemoglobin;
establishing the number of erythrocytes;
determination of clotting indicators

Before the initial stage of the procedure, carry out:

aspirin treatment;
curantile therapy;

After bloodletting, these drugs are also used. Initial treatments include:

reopolyglucin administration;
heparin administration

Once every two days - the time of the meeting. Cytopheresis is a modern method of treatment. The mechanism of cytopheresis:

purification filtration apparatus;
vein catheterization;
screening out a part of red blood cells

Treatment of secondary polycythemia is treatment of the underlying cause of the disease. Intensive oxygen treatment is prescribed for symptoms of oxygen starvation. The infectious type of polycythemia requires the following treatment:

elimination of infection;
antibiotics;
intravenous fluids

The prognosis indicator is timely therapy. Polycythemia of the true type is difficult. The reason is a long course of blood transfusion.

Complications of true polycythemia:

thromboembolism;
the development of a stroke;
development of hypertension

The forecast data depends on the following factors:

primary etiology;
early diagnosis;
correct therapy

I. Definition. Polycythemia is an increase in the total number of red blood cells. The increased viscosity of blood in polycythemia is caused by an increase in the number of red blood cells. A. Polycythemia. Polycythemia in a newborn is defined as an increase in central venous hematocrit up to 65% or more. The clinical significance of this hematocrit value is determined by the curvilinear dependence of the whole blood viscosity on the number of circulating erythrocytes (hematocrit). With an increase in hematocrit of more than 65%, blood viscosity grows exponentially.

B. Increased blood viscosity. An increase in blood viscosity is the direct cause of the onset of pathological symptoms in most newborns with polycythemia. The reason for the increase in blood viscosity is not only an increase in hematocrit, it can be determined or aggravated by other factors. Therefore, the terms "polycythemia" and "high blood viscosity" are not synonymous. Although most children with polycythemia also have increased blood viscosity, this is not always a necessary combination.

II. Pathophysiology. Clinical symptoms of polycythemia in newborns are due to local manifestations of increased blood viscosity: tissue hypoxia, acidosis, hypoglycemia, the formation of microthrombi in the vessels of the microvasculature. The central nervous system, kidneys, adrenal glands, lungs, heart and gastrointestinal tract are most commonly affected. The severity of clinical manifestations is determined by the interaction of cohesion forces in whole blood. These forces are called "shear stress" and "shear rate", which is a measure of blood flow velocity. Cohesive forces act in whole blood and their relative contribution to the increase in blood viscosity in newborns depends on the following factors:

A. Hematocrit. An increase in hematocrit is the most important factor in increasing blood viscosity in newborns. High hematocrit may be associated with an increase in the absolute number of circulating red blood cells or a decrease in plasma volume.

B. Plasma viscosity. There is a direct linear relationship between plasma viscosity and the concentration of proteins in it, especially with a high relative molecular weight, such as fibrinogen. In newborns, and especially in premature infants, plasma fibrinogen levels are lower than in adults. Therefore, except in rare cases of primary hyperfibrinogenemia, plasma viscosity does not affect the viscosity of whole blood. Under normal conditions, low plasma fibrinogen levels and the associated low plasma viscosity actually maintain adequate microcirculation in the newborn by improving tissue perfusion and reducing the viscosity of whole blood.

B. Aggregation of erythrocytes. Aggregation of erythrocytes occurs only in areas with a low blood flow velocity, as a rule, in the venous vessels of the microcirculatory bed. Since full-term and premature infants are characterized by low plasma fibrinogen levels, RBC aggregation does not significantly affect the viscosity of whole blood in newborns. Recently, it has been suggested that the use of fresh frozen adult plasma for partial exchange transfusion in newborns can dramatically change the concentration of fibrinogen in the blood and reduce the degree of expected decrease in the viscosity of whole blood in the microvasculature.

D. Deformation of the erythrocyte membrane. There is no discernible difference in erythrocyte membrane deformation between adults and term and preterm infants.

III. Frequency

A. Polycythemia. Polycythemia occurs in 2-4% of all newborns; in half of them, it manifests itself clinically. Determining the hematocrit value only in newborns with symptoms of polycythemia leads to a decrease in the data on the frequency of polycythemia.

B. Increased blood viscosity. Increased blood viscosity without polycythemia occurs in 1% of healthy newborns. Among children whose hematocrit is 60-64%, one quarter has increased blood viscosity.

IV. Risk factors

A. Factors affecting the incidence of polycythemia

1. Height above sea level. One of the adaptive responses to living in high mountainous areas is an absolute increase in the number of erythrocytes.

2. Postnatal age. Normally, during the first 6 hours of life, fluid moves from the intravascular sector. The maximum physiological increase in hematocrit occurs by 2-4 hours of life.

3. The work of an obstetrician. Delaying clamping of the umbilical cord for more than 30 seconds or pressing it out, if this practice is common, leads to an increase in the frequency of polycythemia.

4. High-risk childbirth. High-risk childbirth often leads to the development of polycythemia in the newborn.

B. Perinatal factors

1. Increased erythropoiesis in the fetus. An increase in the level of erythropoietin is the result of the direct action of intrauterine hypoxia or dysregulation of its production.

and. Placental insufficiency

(1) Maternal hypertension (pre-eclampsia, eclampsia) or primary neovascular disease.

(2) Placental abruption (chronic recurrent).

(3) Prolongation of pregnancy.

(4) Congenital blue heart disease.

(5) Delayed intrauterine development of the fetus.

b. Endocrine Disorders. An increase in oxygen consumption is a putative mechanism for the onset of fetal hypoxia and stimulation of erythropoietin production against the background of hyperinsulinism or hyperthyroxinemia.

(1) Newborns from mothers with diabetes mellitus (polycythemia rate over 40%).

(2) Newborns from mothers with pregnancy diabetes (polycythemia rate over 30%).

(3) Congenital thyrotoxicosis.

(4) Congenital adrenal hyperplasia.

(5) Beckwith-Wiedemann syndrome (secondary hyperinsulinism).

in. Genetic defects (trisomies 13, 18 and 21).

2. Hypertransfusion. Factors that increase placental transfusion at birth can lead to the development of hypervolemic normocythemia in the child, which, as the physiological redistribution of fluid in the body, turns into hypervolemic polycythemia. Massive placental transfusion can cause hypervolemic polycythemia immediately after birth, which manifests itself in a child with acute clinical symptoms. Factors that increase placental transfusion include the following:

and. Late clamping of the umbilical cord. The placental vessels contain up to 1/3 of the total blood volume of the fetus, half of which returns to the child in the first minute of life. The representative volume of circulating blood in full-term newborns, depending on the timing of cord clamping, varies as follows:

(1) after 15 s - 75-78 ml / kg

(2) after 60 s - 80-87 ml / kg

(3) after 120 s - 83-93 ml / kg

b. Gravity Positioning the newborn below the level of the placenta (more than 10 cm) increases the placental transfusion through the umbilical vein. Raising the newborn 50 cm above the placenta level prevents any transfusion.

in. Administration of drugs to the mother. Drugs that increase uterine contractility, in particular oxytocin, do not significantly change the effect of gravity on placental transfusion during the first 15 seconds after birth. However, with a later clamping of the umbilical cord, blood flow to the newborn increases, reaching a maximum by the end of the 1st minute of life.

d. Caesarean section. With a caesarean section, the risk of placental transfusion is usually lower if the umbilical cord is clamped early, as in most cases there are no active uterine contractions and gravitational forces are involved.

e. Feto-fetal transfusion. Feto-fetal transfusion (parabiosis syndrome) occurs in identical twins in 15% of cases. The recipient twin at the venous end of the anastomosis develops polycythemia; the twin donor located at the arterial end of the anastomosis has anemia. Simultaneous determination of the hematocrit values \u200b\u200bof venous blood after birth reveals a difference of 12-15%. Both children are at risk for fetal or neonatal death.

e. Maternal fetal transfusion. Approximately 10-80% of healthy newborns receive a small amount of maternal blood during labor. With the help of the "reverse" Kleihauer-Betke test, the "erythrocytes-shadow" of the mother can be detected in the blood smear of the newborn. With massive transfusion, the test remains positive for several days,

g. Intranatal asphyxia. Prolonged intrauterine hypoxia leads to an increase in the volumetric blood flow in the umbilical cord towards the fetus until it is clamped.

V. Clinical manifestations

A. Symptoms and signs. The clinical symptoms of polycythemia are nonspecific and reflect the local effect of increased blood viscosity in a limited area of \u200b\u200bthe microvasculature. The disorders listed below may occur outside of polycythemia or high blood viscosity and should be considered in the differential diagnosis.

1. Central nervous system. Changes in consciousness, including lethargy, and decreased motor activity or increased excitability are noted. Hypotension in the proximal muscle groups, instability of muscle tone, vomiting, seizures, thrombosis, and cerebral infarction may also occur.

2. Respiratory and circulatory organs. Respiratory distress syndrome, tachycardia, and congestive heart failure with low cardiac output and cardiomegaly are possible. Primary pulmonary hypertension may occur.

3. Gastrointestinal tract. Feeding intolerance, bloating, or necrotizing enterocolitis are observed.

4. Genitourinary system. Oliguria, acute renal failure, renal vein thrombosis, or priapism may develop.

5. Metabolic disorders. There are hypoglycemia, hypocalcemia, hypomagnesemia.

6. Hematological disorders. Possible hyperbilirubinemia, thrombocytopenia, or reticulocytosis (only with increased erythropoiesis).

B. Laboratory research

1. Venous (not capillary) hematocrit. Polycythemia develops with a central venous hematocrit of 65% or more.

2. The following screening tests can be used:

and. Cord blood hematocrit of more than 56% indicates polycythemia.

b. Hematocrit of capillary blood from a warmed heel of more than 65% indicates polycythemia.

in. If, using the table of normal values \u200b\u200bfor blood viscosity, it is established that its value in this child is 2σ or more higher than the norm, this means that he has polycythemia.

Vi. Treatment. Treatment of a newborn with polycythemia is based on the severity of clinical symptoms, the age of the child, the value of the central venous hematocrit and the presence of concomitant diseases.

A. Newborns without clinical symptoms of polycythemia. In most cases, expectant tactics and observation are warranted. The exception is newborns with central venous hematocrit of more than 70%, who are shown partial exchange plasma transfusion. A thorough clinical examination is necessary to detect microsymptoms of polycythemia or high blood viscosity. However, the absence of even microsymptoms in the child does not exclude the risk of neurological complications in the long term.

B. Newborns with clinical symptoms of polycythemia. With a central venous hematocrit of 65% or more at any age, partial exchange plasma transfusion is indicated. If in the first 2 hours of life the central venous hematocrit in a child is 60-64%, carefully monitor the level of hematocrit; decide on a partial exchange transfusion of plasma taking into account the expected physiological redistribution of fluid in the body and a further decrease in the volume of circulating plasma. The technique for performing partial exchange plasma transfusion is described in Chapter 17. The effectiveness of partial exchange plasma transfusion in newborns with polycythemia remains controversial.

Vii. Forecast. Long-term results of treatment of newborns with polycythemia or increased blood viscosity using partial exchange plasma transfusion are as follows:

A. There is a causal relationship between the operation of partial exchange plasma transfusion and an increase in the incidence of dysfunction of the gastrointestinal tract and ulcerative necrotizing enterocolitis.

B. Randomized controlled prospective studies of the development of newborns with polycythemia and increased blood viscosity indicate that partial exchange transfusion of plasma reduces, but does not completely eliminate the risk of neurological disorders in the long term.

B. Neonates with asymptomatic polycythemia have an increased risk of developing neurological disorders.

D. Neurological disorders observed in the long-term in newborns with polycythemia who have not undergone partial exchange plasma transfusion include speech disorders, delay in the acquisition of skills in performing rough and precise movements, and general developmental delay.

Regional maternity hospital

"APPROVED"

Chief physician of the ORD

M. V. Mozgot

"________" _____________________ 2007

Diagnosis and treatment of polycythemia in newborns

Yaroslavl - 2009

List of abbreviations

IUGR - intrauterine growth retardation

BCC - the volume of circulating blood

ChIG - partial isovolemic hemodilution

YANEK - necrotizing ulcerative enterocolitis

Ht - hematocrit

Hb - hemoglobin

Pathophysiology

Risk factors for developing polycythemia

An increase in erythropoiesis in the fetus is secondary due to intrauterine hypoxia:

endocrine disorders are associated with increased oxygen metabolism in fetal tissues. Includes congenital thyrotoxicosis, Beckwith-Wiedemann syndrome, the presence of diabetic fetopathy with inadequate glucose control;

genetic disorders (trisomies 13, 18 and 21).

Hypertransfusion:

delayed clamping of the umbilical cord. A delay in clamping the umbilical cord for more than 3 minutes after delivery leads to an increase in BCC by 30%;
delaying cord clamping and exposure to uterotonic drugs lead to an increase in blood outflow to the fetus (in particular, oxytocin);
force of gravity. Depends on the position of the newborn in height relative to the mother's body before clamping the umbilical cord (if below the placenta level by more than 10 cm);
fetal-fetal transfusion syndrome (about 10% of monozygous twins);
home birth;
intranatal asphyxia leads to a redistribution of blood from the placenta to the fetus.

Clinical manifestations

Discoloration of the skin:

crimson, bright red skin color
may be normal or pale.

From the side of the central nervous system:

changes in consciousness, including lethargy,

decreased physical activity,

increased excitability (jitteriness),

difficulty sucking

convulsions.

Respiratory and cardiovascular disorders:

respiratory distress syndrome,

tachycardia,

muted tones,

congestive heart failure with low cardiac output and cardiomegaly

primary pulmonary hypertension may occur.

Gastrointestinal tract:

feeding problems,

bloating

ulcerative-necrotizing enterocolitis (often not associated with polycythemia itself and occurs when colloids are used as a replacement solution during partial hemodilution, which cannot be said about crystalloids).

Genitourinary system:

acute renal failure

priapism in boys - pathological erection due to sludge of red blood cells.

Metabolic disorders:

hypoglycemia,

hypocalcemia,

hypomagnesemia.

Hematological disorders:

hyperbilirubinemia,

thrombocytopenia,

hypercoagulability with the development of disseminated intravascular coagulation,

reticulocytosis (only with increased erythropoiesis).

The lecture was given by: MD, prof. Pyasetskaya N.M., dept. Neonatology on the basis of the Ukrainian Children's Specialized Hospital of the Ministry of Health of Ukraine "OKHMATDET".

Polycythemia of the newborn

Polycythemia is a malignant increase in the number of blood germ cells: erythroid to a greater extent, platelet and neutrophilic to a lesser extent.

ICD-10 code: R61, R61.1

Clinical diagnosis:

Neonatal polycythemia (erythrocytosis, primary polycythemia, true) is diagnosed when:

Ht veins. (Venous hematocrit)\u003e 70% or venous Hb\u003e 220 g / l.

An example of a diagnosis: Primary polycythemia with severe erythrocytosis, thrombocytosis and leukocytosis, II degree. (erythremic stage). Hepatosplenomegaly. Vascular thrombosis.

The incidence is:

2-5% - in healthy full-term newborns,

7-15% - in premature babies.

Polycythemia problem:

reduced transport function of erythrocytes;
oxygen supply to tissues is impaired (Ht veins\u003e 65%).

The causes of polycythemia:

1) Intrauterine hypoxia (increased erythropoiesis):

gestosis of pregnant women;
severe heart disease of the mother;
placental insufficiency of an infant with intrauterine hypotrophy;
postmaturity (additional fluid loss);

2) Lack of oxygen delivery (secondary polycythemia of the newborn):

impaired ventilation (lung disease);
congenital blue heart defects;
congenital methemoglobinemia;

3) Risk group for the development of neonatal polycythemia in newborns:

Diabetes mellitus in the mother;
Late clamping of the umbilical cord (\u003e 60 sec);
Feto-fetal or maternal-fetal transfusion;
Congenital hypothyroidism, thyrotoxicosis;
Down syndrome;
Wiedemann-Beckwith syndrome;

Classification of polycythemia in newborns:

1) Neonatal polycythemia:

2) Primary polycythemia:

Polycythemia vera;
Erythrocytosis (benign familial polycythemia of the newborn);

3) Secondary polycythemia - the result of insufficient oxygen delivery (promotes the synthesis of erythropoietin, which accelerates erythropoiesis and increases the number of red blood cells), or a failure in the hormone production system.

A. Oxygen deficiency:

Physiological: during intrauterine development; low oxygen content in the inhaled air (high altitude).
Pathological: impaired ventilation (lung disease, obesity); arteriovenous fistulas in the lungs; congenital heart disease with an intracardiac shunt from left to right (tetrad of Fallot, Eisenmenger complex); hemoglobinopathies: (methemoglobin (congenital or acquired); carboxyhemoglobin; sulfhemoglobin; hemoglobinopathies with a high affinity of hemoglobin for oxygen; lack of 2,3-diphosphoglycerate mutase in erythrocytes.

B. Enhanced erythropoiesis:

a) on the part of the kidneys: Wilms tumor, hypernephroma, renal ischemia, vascular diseases of the kidneys, benign neoplasms of the kidneys (cysts, hydronephrosis);

b) from the adrenal glands: pheochromocytoma, Cushing's syndrome, congenital adrenal hyperplasia with primary aldosteronism;

c) from the liver: hepatoma, focal nodular hyperplasia;

d) from the side of the cerebellum: hemangioblastoma, hemangioma, meningioma, hepatocellular carcinoma, liver hemangioma;

e) from the side of the uterus: leiomyoma, leiomyosarcoma.

a) the use of testosterone and related steroids;

b) the introduction of growth hormone.

4) False (relative, pseudocythemia).

Stages of neonatal polycythemia:

I Art. (initial) - the clinical picture is erased, the disease is sluggish. The first stage can last up to 5 years. It is possible to suspect the disease only with a laboratory blood test, in which there is a moderate erythrocytosis. Objective data are also not very informative. The spleen and liver are slightly enlarged, but this is not a pathognomonic sign of this disease. Complications from internal organs or blood vessels are extremely rare.

II Art. (proliferation) - the clinic is characterized by the height of the disease. There is a pletora, hepatosplenomegaly, depletion of the body, manifestation of thrombosis, convulsions, tremor, dyspnea. In the general analysis of blood - erythrocytosis, thrombocytosis, neutrophillosis with a shift to the left, or panmyelosis (an increase in the amount of all blood elements). In the blood serum, the content of uric acid rises (norm \u003d up to 12 years - 119-327 μmol / l), which is synthesized in the liver and excreted by the kidneys. It circulates in blood plasma in the form of sodium salts.

III (emaciation, anemic) - clinical signs in the form of pletora, hepatosplenomegaly, general weakness, significant weight loss. At this stage, the disease becomes chronic and myelosclerosis may occur.

Syndromes that are accompanied by an increased level of Ht veins.

Blood hyperviscosity (not synonymous with polycythemia) is the result of increased levels of fibrinogen, IgM, osmolarity and blood lipids. Addiction with polycythemia becomes exponential when Htwen exceeds 65%.
Hemoconcentration (relative polycythemia) is an increased level of hemoglobin and hematocrit due to a decrease in plasma volume due to acute dehydration of the body (exsicosis).

General Clinic for Polycythemia:

Pletora (with primary polycethemia) is a general plethora of the body. There is a reddening of the face (becomes purple), a strong, high pulse, "beating in the temples", dizziness.
Insufficient filling of capillaries (acrocyanosis).
Dyspnea, tachypnea.
Depression, drowsiness.
Weakness in sucking.
Persistent tremor, muscle hypotonia.
Convulsions.
Bloating.

Complications (clinical conditions associated with polycythemia and hemoconcentration syndrome (thickening) of the blood):

Pulmonary hypertension with the development of PFC syndrome (persistent fetal circulation).
Increased systemic blood pressure.
Venous congestion in the lungs.
Increased tension on the myocardium.
Hypoxemia.
Metabolic disorders (hyperbilirubinemia, hypocalcemia, hypomagnesemia).
Increased glucose utilization (hypoglycemia)
Hepatomegaly.
Intracranial hemorrhage, convulsions, apnea.
Renal vein thrombosis, acute renal failure (acute renal failure), oliguria.
Ulcerative necrotizing enterocolitis.
Decreased blood circulation in the gastrointestinal tract, kidneys, brain, myocardium.

Diagnostics.

Laboratory data:

Ht veins
general blood analysis

It should be remembered that 4-6 hours (sometimes earlier) after birth, hemoconcentration necessarily occurs (rise in hematocrit, hemoglobin, leukocytes) due to certain physiological mechanisms.

Additional examinations:

platelets (thrombocytopenia),
blood gases
blood sugar (hypoglycemia),
bilirubin (hyperbilirubinemia),
urea,
electrolytes,
x-ray of the lungs (with RDS).

If necessary (determination of blood hyperviscosity), determine fibrinogen, IgM, blood lipids, calculate the osmolarity of the blood.

Differential diagnosis of true neonatal polycythemia, true secondary polycythemia due to hypoxia and pseudo-polycythemia (relative).

True neonatal polycythemia:

There is granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of erythrocytes is increased;
The regulator of erythropoiesis (erythropoietin) is normal or decreased;

True secondary polycythemia due to hypoxia:

The mass of erythrocytes is increased;
Plasma volume unchanged or reduced;
The regulator of erythropoiesis (erythropoietin) is increased;
Decreased or normal arterial oxygen saturation.

Pseudo-polycythemia:

No granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of red blood cells is unchanged;
Plasma volume is reduced;
The erythropoiesis regulator (erythropoietin) is normal;
Normal arterial oxygen saturation.

Treatment of polycythemia.

1) General activities:

Ht vein level control:

a) with Ht veins 60-70% + no clinical signs \u003d control after 4 hours

b) with Ht veins\u003e 65% + clinical signs \u003d normovolemic hemodilution or partial exchange transfusion (exfusion).

Repeated control of Ht veins: 1, 4, 24 hours after hemodilution or partial exchange transfusion

Normovolemic hemodilution:

Purpose: to reduce the level of Ht veins to 50-55% by diluting the blood (more often this method is used in the presence of dehydration).

Partial exchange transfusion:

Formula for calculating the required volume (ml) of exfusion - infusion or hemodilution:

V (ml) \u003d BCC child (ml / kg) * (Ht child - Ht desired) / Ht child, where

V (ml) - volume of partial exchange transfusion (infusion)

Ht desired ≈ 55%

BCC of a full-term baby - 85-90 ml / kg

BCC of a premature baby - 95-100 ml / kg

Child's HT - 71%;

Ht desired - 55%;

BCC of a child - 100 ml / kg;

Child's body weight - 3 kg

V (ml) \u003d 100 x 3 x (71% - 55%) 300 ml x 16% / 71% \u003d 67.6 ml. or 17 ml. x 4 times *

Solutions that can be used for hemodilution and partial exchange transfusion:

physiological solution (0.85% sodium chloride solution);
ringer's solution or Ringer's lactate;
colloidal solutions based on hydroxyethyl starch (HES) - 6%, 10% Refortan solution (the indication for the use of this solution is hemodilution, correction of hemodynamic disturbances, improvement of rheological properties of blood, and others). There is little experience in neonatology.

Human plasma (FFP) should not be used.

With latent (asymptomatic) polycythemia, the risk of neurological disorders increases.

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Polycythemia in children and adults

A blood disorder accompanied by an increased volume of erythrocyte germ cells is called polycythemia. Depending on the causes, pathology is divided into true (primary) and secondary.

The studied prevalence of polycythemia vera makes it possible to determine as the most dangerous group of middle-aged and elderly people, most often men. Every year, 4 - 5 primary cases are registered for every million of the population.

In newborns, polycythemia may occur secondarily in response to tissue oxygen hypoxia.

Varieties of the disease

The types of the disease differ in the severity of the course and the main connection with the mechanism of damage to the hematopoietic organs.

Polycythemia vera is always a manifestation of tumor-like proliferation of cells, precursors of normal erythrocytes.
Secondary polycythemia is formed under the influence of various diseases that cause "thickening" of the blood.

It can be:

dehydration factor (loss of fluid from frequent and profuse vomiting with cholera, poisoning, diarrhea, large burn surface);
hypoxia (lack of oxygen) caused by climbing the mountains, heat, increased sweating with fever.

A secondary condition is a clinical manifestation of another disease, such as salmonellosis or dysentery. At the same time, the total mass of erythrocytes in the blood of patients remains normal.

In hot climates, a person loses a lot of fluids through sweat, in the absence of drinking, polycythemia will occur

A decrease in the plasma part leads to a relative shift towards higher viscosity. Treatment of this pathology is always associated with the compensation of fluid in the body. This leads to a complete restoration of the ratio of erythrocytes and plasma.

Mechanisms of development of polycythemia

Dehydration and oxygen deficiency in tissues force the human body to compensate for the production of hemoglobin due to the additional synthesis of red blood cells (cells that carry oxygen molecules). At the same time, the erythrocytes produced by the bone marrow have the correct shape, volume, and have all the functions.

In contrast to this process, polycythemia vera is accompanied by mutation of stem cells in the red bone marrow. The synthesized cells belong to the precursors of erythrocytes, do not correspond to the size, are not needed by the body in large quantities.

The relationship of tumor growth with two cell populations has been proven:

the first develops independently (autonomously) from defective progenitors due to genetic mutation;
the second depends on the effects of erythropoietin, a hormone produced by the kidneys, which controls not only the production of the number of red blood cells, but also their correct step-by-step differentiation.

The “triggering” of the erythropoietin activation mechanism explains the attachment of secondary polycythemia to the tumor process.

A huge amount of unnecessary blood cells leads to its thickening and provokes increased blood clots. The accumulation in the spleen, which does not have time to destroy the formed elements, leads to an increase in its mass, elongation of the capsule.

Main causes and risk factors

Genetic inherited mutations are believed to be the main cause of primary polycythemia. Found genes responsible for the synthesis of erythrocytes and exhibiting special sensitivity to erythropoietin. Such a pathology is considered familial, since it is found in relatives. One of the variants of genetic changes is the pathology of genes, when they begin to capture more oxygen molecules, but do not give it to the tissues.

Polycythemia develops secondarily as a result of long-term chronic diseases that stimulate an increased level of erythropoietin. These include:

Related article: Why can the average platelet volume be increased?

chronic respiratory diseases with impaired patency (obstructive bronchitis, asthma, pulmonary emphysema);
increased pressure in the pulmonary artery with heart defects, thromboembolism;
heart failure as a result of ischemia, decompensation of defects, the consequences of arrhythmia;
ischemia of the kidneys with atherosclerosis of the leading vessels, cystic degeneration of tissues.

In oncological diseases, varieties of malignant tumors have been found that activate the production of erythropoietin:

liver carcinoma;
renal cell carcinoma;
swelling of the uterus;
neoplasm of the adrenal glands.

Polycythemia in smokers occurs when oxygen in the inhaled air is replaced by carbon oxides and other toxic substances.

Risk factors can be:

stressful situations;
long-term stay in high mountains;
professional contact with carbon monoxide when working without protective masks in a garage, coal mines, electroplating shops.

Clinical manifestations

The symptoms of polycythemia appear in the second stage of the disease. Initial signs can only be detected by laboratory examination. They are often hidden behind the underlying disease.

Patients complain of frequent headaches, dizziness, a feeling of "heaviness" in the head.
Itching of the skin begins gradually and takes on a painful character. It is explained by the increased release of histamine-like substances by mast cells. Typical increased itching while taking a shower or bath, when washing.
Changes in skin color - patients are distinguished by a red, swollen face, the hands become crimson with a bluish tinge.
Painful sensations in the fingers occur when touching objects.
Significant increase in systolic blood pressure (up to 200 mm Hg and above) is characteristic.
Due to the enlargement of the spleen, pain appears in the left hypochondrium. The liver also reacts, on examination, a protruding edge is found.
Pain in the bones (thighs, along the ribs).
Increased fatigue, tendency to acute infections.
In the final stage, signs of bleeding appear: bruises on the body, bleeding from the nose, gums.

One symptom is facial flushing and injected sclera

The disease can be detected upon detection of increased thrombus formation and clinical manifestations of stroke, acute heart attack, manifestations of embolism in the mesenteric vessels (abdominal pain).

Flow stages

The true form is characterized by 3 stages of the course of the disease:

the onset of the disease or the height - according to clinical signs, only altered laboratory parameters can be detected (erythrocytosis, increased hemoglobin and hematocrit), the patient does not present complaints;
the second - all symptoms appear, characteristic signs of a violation of hematopoiesis;
the third or final - there is anemia due to depletion of the bone marrow, internal and external bleeding, a sharp increase in the spleen and liver, manifestations of hemorrhage in the brain are added to the symptoms of the second period.

Treatment of polycythemia is based on syndromic treatment, depending on the identified period of the disease.

The development of pathology in children

In childhood, polycythemia is most often found in newborns (neonatal form). It is detected in the first 2 weeks of a baby's life. Its cause is the response of the child's body to the transferred oxygen deficiency in the womb due to disturbed placental nutrition.

Twins are especially susceptible to genetic changes. By the cyanosis of the baby's skin, he is suspected of having heart defects, a violation of the respiratory mechanism, which are accompanied by an increased level of erythrocytes. Hemoglobin increases 20 times.

The stages of the disease in newborns are the same as in adults. The baby, due to soreness and itching, does not allow to touch the skin. In children, other blood growths suffer much faster: thrombocytosis, leukocytosis appear.

The kid does not gain weight, exhaustion worsens the general condition. In the final stage, the production of immune cells stops. The child can die from any infection.

The diagnosis of the disease is based on a laboratory blood test:

When calculating the number of erythrocytes, their number is found from 6.5 to 7.5 x 1012 per liter. The indicator of polycythemia is the excess of the total weight in men 36, in women 32 ml / kg of weight.
At the same time there is leukocytosis, thrombocytosis in the first stages.
In a smear, the laboratory assistant sees a large number of erythrocyte precursors (metamyelocytes).
From biochemical tests, attention is paid to the increase in the activity of alkaline phosphatase.

Modern equipment allows you to quickly and objectively diagnose

Bone marrow analysis provides a definitive diagnosis.

Treatment

It is important to consider the underlying cause of polycythemia in the treatment regimen. If the secondary changes can be compensated, limited, then the therapy of primary tumor cell proliferation is very difficult.

Restrictions on the regimen are not required unless thrombotic complications have occurred.

In the diet, it is necessary to provide for a significant amount of fluid and the restriction of foods that increase the synthesis of hemoglobin, containing a lot of iron. These include: chicken, beef, turkey, liver in any form, fish, from cereals - buckwheat and millet, chicken eggs. Fatty broths are not shown. Dairy products are recommended.

Medicines are used that suppress the activity of the bone marrow (hydroxycarbamide, hydroxyurea). Cytostatics include Mielosan, Myelobromol.

Bloodletting allows for a short period to slow down the pathological effect of the mass of erythrocytes

A decrease in hematocrit with blood withdrawal up to 46% is acceptable. Before the first procedure, blood clotting indicators are studied, and Aspirin and Curantil are prescribed to prevent the risk of platelets sticking together. Possible drip introduction of Reopolyglyukin, Heparin.

The volume of a one-step intake is up to 500 ml (with concomitant heart failure - 300). The procedures are carried out in a course every other day.

Cytopheresis - blood purification using special filters. Allows to retain some of the red blood cells and return the patient's own plasma.

Traditional therapy

Treatment with folk remedies for polycythemia vera is very problematic, since no such method has yet been found. Therefore, doctors recommend that patients refrain from using any advice from healers.

The most popular among them are decoctions of cranberries and sweet clover herbs. Medicinal tea is prepared from them and drunk during the day.

Forecast

The prognosis of the primary form of the disease is very unfavorable: without treatment, patients live for two years, no more. Death comes from thrombosis or bleeding with brain damage.

Bloodletting and other modern methods of therapy have made it possible to extend the life of patients by 15 years or more.

If there are changes that are unexpectedly detected in blood tests, do not be immediately scared. Pre-examination, first of all, will exclude the possibility of improper preparation for analysis (after stress, night shift, food intake). This can significantly affect the results. If the diagnosis is confirmed, only a quick start of treatment will provide the necessary assistance.

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Polycythemia

Polycythemia is a disease characterized by an increase in the volume of red blood cells in the bloodstream. This disease can be either primarily caused or secondary to the impact of certain underlying causes. Both primary and secondary polycythemia are quite formidable diseases that can lead to serious consequences.

So primary or polycythemia vera is manifested when a tumor substrate appears in the bone marrow and an increased production of erythrocytes. Therefore, at advanced stages, this lesion increases in size and displaces all other substrates from the bone marrow - the progenitors of future blood cells.

Secondary polycythemia occurs in completely different situations, but one of the leading is general hypoxia (oxygen starvation) of the body. Thus, secondary polycythemia is an indicator of certain pathological processes in the body, arising as a compensatory reaction.

Polycythemia vera

Polycythemia vera is a disease with a purely neoplastic genesis. Fundamental in this disease is that stem cells are affected in the red bone marrow, or rather the progenitor cells of the blood cells (also called pluripotent stem cells). As a result, the number of erythrocytes and other corpuscles (platelets and leukocytes) increases sharply in the body. But since the body is adapted to a certain rate of their content in the blood, any excess of the limits will entail certain disturbances in the body.

Polycythemia vera is quite malignant and difficult to treat. This is due to the fact that it is almost impossible to influence the main cause of polycythemia vera - a mutated stem cell with high mitotic activity (ability to divide).

Pletoric syndrome will be a striking and characteristic sign of polycythemia. It is due to the high content of red blood cells in the stream. This syndrome is characterized by a purple-red skin color with severe itching.

Polycythemia vera has three main stages, which are located according to the activity of the process. The first stage is the peak stage. At this stage, the first changes in the bone marrow will form, and altered areas of hematopoiesis will form. The heat stage is almost impossible to detect clinically. Most often at this stage, the diagnosis of polycythemia vera is made randomly, for example, when doing blood tests to diagnose another disease.

After the peak stage, the stage of clinical manifestations follows: here all the clinical signs of this disease, pletora syndrome, itching, and enlargement of the spleen appear. After the stage of clinical manifestations, the final stage will appear - anemic. With her, all the same clinical signs will be revealed, plus diagnostic symptoms of bone marrow "devastation" will be added to them (due to constant bone marrow hyperplasia).

It is important to note that polycythemia vera is a formidable disease due to its complications. An increased number of red blood cells and platelets will lead to increased thrombus formation and the development of thrombotic lesions in the body. In addition, overall blood pressure rises, which can lead to persistent hypertension and hemorrhagic strokes, followed by intracranial hemorrhage and death.

Causes polycythemia

With polycythemia, the main manifestation will be an increase in the bloodstream, for various reasons, the number of erythrocytes. The reasons for this mechanism will depend on the type of polycythemia. Distinguish between absolute and relative polycythemia.

With absolute polycythemia, there is a direct increase in the volume of erythrocytes in the bloodstream. Absolute polycythemia includes polycythemia vera, polycythemia in hypoxic conditions and obstructive pulmonary lesions, hypoxia associated with lesions of the kidneys and adrenal glands. In all these conditions, an increased synthesis of erythrocytes occurs.

In polycythemia vera, erythrocytes intensively synthesize tumor hyperplastic areas of the bone marrow, hypoxia will cause a reciprocal increase in the number of erythrocytes in the blood, and with certain kidney damage, the synthesis of erythropoietin, the main hormone responsible for starting the formation of new erythrocytes, may increase.

With relative polycythemia, erythrocyte volumes will increase due to a decrease in plasma volume. Normally, plasma is about 5% more than blood cells. With the loss of plasma, this ratio is violated, the plasma becomes smaller. The main paradox is that with relative polycythemia, the number of erythrocytes literally does not change - it remains within normal limits. But due to a decrease in blood plasma in the ratio of plasma: shaped elements, there are more of them - their "relative" number increases.

So the relative polycythemia includes such infectious diseases as cholera, dysentery and salmonellosis. With them, severe vomiting and diarrhea are observed, which leads to the loss of the body of significant internal reserves of water, including plasma. In addition, burns can lead to a decrease in plasma volume and the development of relative polycythemia, as well as exposure to high temperatures, which will cause increased sweating.

It is also necessary to pay special attention to the two most common causes of polycythemia: tumor lesion of the red bone marrow and the effect of hypoxia on the synthesis of red blood cells.

Tumor damage to the red bone marrow is fundamental for the development of primary or polycythemia vera. With this type of polycythemia, a mutation occurs at the level of the stem cell genome, and it begins to divide uncontrollably, creating its own new tumor clones. It is important to note here that these pluripotent stem cells are the "alpha and omega" of all future blood cells: erythrocytes, leukocytes and platelets. These pluripotent cells undergo a certain differentiation during their growth and form three main areas of hematopoiesis according to the number of formed elements: erythrocyte, platelet and leukocyte. Then, from each sprout, future shaped elements are gradually born.

But in polycythemia vera, the pluripotent stem cell is already with a genetic defect, and it synthesizes exactly the same defective subsequent progenitor cells of the hematopoietic sites. As a result, these cells are part of the hematopoietic sprouts and, like their progenitors, divide intensively, creating a huge number of shaped elements. Thus, two pathological processes are formed in polycythemia vera - an excess of normal levels of erythrocytes and simultaneous hyperplasia (increase in size) of the affected areas of hematopoiesis.

The effect of hypoxia on the body has a number of pathological phenomena, among which the development of secondary polycythemia occupies an important place. The cause of secondary polycythemia during hypoxia is explained by the fact that the body is trying to compensate for the lack of oxygen in the body by synthesizing new red blood cells. This is due to the effect of hypoxia on the kidneys, during which the latter produces a special substance - erythropoietin. It is erythropoietin that triggers the processes of differentiation of the pluripotent stem cell into reticulocytes (precursors of erythrocytes) and the further formation of new erythrocytes. Therefore, when hypoxia affects hematopoiesis, the following connection can be traced: the stronger its effect on the body, the more erythropoietin will be synthesized by the kidneys, and the stronger the erythrocyte region of the bone marrow will work, synthesizing new red blood cells.

Polycythemia symptoms

The main and, perhaps, the most important symptom of polycythemia will be the so-called "pletora syndrome". This syndrome is caused by an increase in the volume of all blood cells and general plethora.

The basis of plethoric syndrome will be the complaints of the patients themselves, as well as disorders that can be determined using objective research.

Among the complaints of patients, the main indicators of plethoric syndrome will be persistent headaches, which will alternate with dizziness. In addition, plethoric syndrome will always accompany patients' complaints of itching. Its occurrence is explained by the fact that with polycythemia vera, there is a massive synthesis by mast cells of special substances - prostaglandins and histamines, which, acting on histamine receptors, will lead to the appearance of severe, sometimes even unbearable skin itching. By the way, one of the classic and characteristic features of polycythemia will be the special nature of this skin itch - it can increase several times, after skin contact with water (when taking a bath, shower, or even with simple washing). But it is important to know that pletora is a symptom of purely polycythemia vera. With secondary polycythemia, the color of the skin will not undergo such significant changes.

In addition to the complaints described above, patients will also indicate changes in the hands. These changes are called erythromelalgia. The skin of the hands will have a characteristic “red-cyanotic” color. In addition, a change in the color of the hands and fingers will be accompanied by severe pain in the affected areas, which will occur with each contact with any surface. The cause of this condition is the same as for plethoric itching - the production of high amounts of histamines.

Objectively, it is possible to determine the presence of a specific color in patients with polycythemia - the skin will be bluish-red, sometimes even cherry-colored. There will also be significant changes in the cardiovascular system. The most characteristic of them will be: excess blood pressure and the development of thrombosis. A characteristic sign of changes in blood pressure in polycythemia will be an increase in systolic blood pressure above 200 mm Hg.

The second syndrome in polycythemia is myeloproliferative syndrome. This symptom complex is more typical for true or primary polycythemia. This syndrome is accompanied by an enlargement of the spleen or liver. Its main reason is that the spleen in the body performs the function of the so-called "depot" or "storehouse" for red blood cells. Normally, in the spleen, erythrocytes ending their life cycle are destroyed. But since in polycythemia the number of red blood cells can sometimes exceed the norm by tens of times, the number of red blood cells accumulating in the spleen is enormous. As a result, spleen tissue hyperplasia and its enlargement occur. To an increase in the spleen in myeloproliferation syndrome, patients' complaints of weakness, increased fatigue, as well as pain in the tubular bones and in the left hypochondrium will also be added. Pain symptoms are explained precisely by the proliferation of the hematopoietic areas altered by the tumor and the hyperplastic spleen.

In addition, with any polycythemia, symptoms may appear that are associated with an increase in blood viscosity. These include small and large thrombosis, the development of strokes and heart attacks, as well as the separation of a thrombus and the subsequent development of pulmonary embolism.

But in addition to the symptoms of polycythemia described above, directly related to the pathogenesis of this disease, namely, an increase in the number of red blood cells in the bloodstream and the changes following this state, there are also symptoms - signs of major diseases that led to the development of secondary polycythemias. Such symptoms can be cyanosis (acrocyanosis and widespread cyanosis) at the root causes of secondary polycythemia from the respiratory system (most often chronic obstructive lesions of the pulmonary system can lead to its appearance) and the effects of general hypoxia of the body. Symptoms of impaired renal function or tumor lesions may also be detected, which can also cause secondary polycythemia.

In addition, one should not forget about the effect of infectious agents on the main mechanism of development of polycythemia. The main possible signs in infectious secondary polycythemias will be profuse diarrhea and vomiting, which will lead to a sharp decrease in plasma volume, which means a nonspecific increase in the number of red blood cells.

Polycythemia of the newborn

The first signs of the development of polycythemia can also be observed in newborns. Polycythemia of a newborn occurs as a response of the child's body to intrauterine hypoxia that it has suffered, which could develop due to placental insufficiency. In response, the infant's body, trying to correct hypoxia, begins to synthesize an increased number of erythrocytes. Exactly the same oxygen deficiency as a triggering factor of neonatal hypoxia can be observed in the presence of “blue” congenital heart defects or pulmonary diseases in a newborn.

In addition to respiratory-related polycythemia, neonates, as well as adults, can develop polycythemia vera. Twins are especially at risk.

This disease occurs in the first weeks of a newborn's life and its first signs will be a significant increase in hematocrit (up to 60%) and an increase in hemoglobin values \u200b\u200bby 22 times.

Neonatal polycythemia has several stages of its clinical course: the initial stage, the proliferative stage, and the wasting stage.

At the initial stage, polycythemia practically does not manifest itself in anything and develops without any clinical manifestations. In addition, it is possible to determine the presence of polycythemia in a child at this stage only by examining the indicators of peripheral blood: hematocrit, hemoglobin and the level of erythrocytes.

The stage of proliferation has a much brighter clinic. At this stage, the child has an enlarged liver and spleen. Plethoric phenomena develop: the skin becomes a characteristic "plethoric-red" shade, the child's anxiety when touching the skin. Thrombosis will be added to plethoric syndrome. In the analyzes there will be a change in the number of platelets, erythrocytes and leukocyte shifts. In addition, the parameters of all blood cells may increase - this phenomenon is called panmyelosis.

At the stage of exhaustion, the child will still have signs of enlargement of the spleen and liver, significant loss of body weight, asthenia and exhaustion will appear.

Such clinical changes are very difficult for the newborn and can lead to irreversible changes and subsequent death. In addition, polycythemia vera of a newborn can lead to sclerosis processes in the bone marrow, since due to the constant proliferation of tumor cells in the bone marrow, normally functioning hematopoietic tissue is displaced and its connective tissue is replaced. In addition, this phenomenon can lead to a disruption in the production of certain types of white blood cells, which are responsible for the immune defense of the child's body. As a result, a newborn can develop severe bacterial infections, which become the cause of their death.

Polycythemia treatment

For the correct treatment of polycythemia, it is important to determine the root cause, which became the trigger for the development of this pathology. It is the difference in the effect on the main triggering factor of polycythemia that will be fundamental in the treatment regimen. So, for example, in case of secondary polycythemia, they are engaged in eliminating its root cause, and in polycythemia vera, they try to influence tumor cells and stop the consequences of increased production of cells - formed elements of blood.

Polycythemia vera is quite difficult to treat. It is rather difficult to influence tumor cells and stop their activity. In addition, age is fundamental in prescribing polycythemia therapy aimed at inhibiting the metabolism of tumor cells. So, for patients with polycythemia vera who are under the age of 50, the appointment of certain drugs is strictly prohibited, they are prescribed only to those patients whose age is over 70 years. Most often, myelosuppressive drugs are used to suppress the tumor process: Hydroxyurea, Hydrea, Hydroxycarbamide.

But in addition to the direct effect on the tumor in the bone marrow, it is also important to fight the consequences of the increased content of blood corpuscles. With a high number of red blood cells, the bloodletting procedure is extremely effective. In addition, this procedure is leading in the treatment of polycythemia vera. The main goal pursued when prescribing bloodletting in polycythemia vera is to reduce the hematocrit to 46%. The unit volume of blood that is usually removed during the procedure corresponds to approximately 500 ml. In the presence of certain pathologies (for example, dysfunction of the cardiovascular system), this volume is reduced to 300-350 ml.

Before the first bloodletting procedure, patients are assigned a number of studies aimed at determining hemoglobin parameters, establishing the exact number of erythrocytes, hematocrit, and determining the coagulation parameters. All these indicators are necessary for the correct calculation of the frequency of the procedure, the volume of blood withdrawn and the calculation of target indicators (especially hematocrit).

Before starting the first bloodletting, the patient is prescribed antiplatelet drugs: Aspirin or Curantil. By the way, the same drugs are prescribed for use within a few weeks after the end of the procedure. Before the bloodletting itself, the patient is also prescribed the introduction of rheopolyglucin with heparin to improve the aggregate state of the blood. Usually, the procedures are carried out in order of 1 every two days.

Another more modern treatment for polycythemia vera is cytopheresis. This procedure consists in the fact that the patient is connected to a special apparatus with purifying filters. By catheterizing the veins of both hands, the patient's circulatory system is closed on this apparatus in such a way that blood from one vein enters the apparatus, passes through filters and returns to the other vein. This apparatus centrifuges the blood entering it and “weeds out” part of the erythrocytes, returning the plasma to the patient. Thus, this apparatus removes excess red blood cells from the bloodstream in patients with polycythemia vera.

As mentioned above, secondary polycythemia is cured when the underlying cause of polycythemia is healed and eliminated. In case of hypoxic underlying causes of polycythemia, intensive oxygen therapy is prescribed, as well as the elimination of the hypoxic factor, if any. In case of polycythemias that have developed as a result of infectious diseases, the infectious agent itself is first eliminated by prescribing antibiotics, and in case of loss of large volumes of fluid, replacement intravenous infusions of colloidal solutions are performed.

The prognosis of polycythemia directly depends on its type and timeliness of treatment. Polycythemia vera is a disease with a more severe prognosis: due to the difficulty in its therapy and the constant increase in the number of erythrocytes, such patients are forced to constantly continue courses of hemoexfusion therapy. In addition, these patients are at high risk of thromboembolic complications, stroke and arterial hypertension. The prognosis of secondary polycythemia depends on the underlying disease. In addition, the effectiveness of its elimination depends on early diagnosis and the appointment of adequate treatment.

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Polycythemia is ... Polycythemia: Symptoms and Treatment

Polycythemia is a chronic condition in which the amount of red blood cells (red blood cells) in the blood increases. Also, with such a pathology, in 70% of patients, the number of platelets and leukocytes changes upward.

The disease does not have a high prevalence - no more than five cases are recorded annually per million of the population. Most often, the disease polycythemia develops in middle-aged and elderly people. According to statistics, males suffer from this pathology five times more often than women. Today we will take a closer look at a condition such as polycythemia, the symptoms and treatment of pathology will be described below.

The reasons for the development of the disease

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

Classification of the disease

There are two groups of ailment:

Polycythemia vera, or Vakez's disease, which in turn is divided into primary (that is, it acts as an independent disease) and secondary (secondary polycythemia develops due to chronic lung diseases, tumors, hydronephrosis, rise to height).

Relative polycythemia (stress or false) - in this condition, the level of red blood cells remains within the normal range.

Polycythemia: symptoms of the disease

Very often, the disease is asymptomatic. Sometimes, as a result of examination for completely different reasons, polycythemia vera may be accidentally detected. Symptoms to watch out for are discussed further.

Expansion of the saphenous veins

With polycythemia, dilated saphenous veins appear on the skin, most often in the neck area. With such a pathology, the skin becomes a reddish-cherry hue, this is especially noticeable on open areas of the body - neck, hands, face. The mucous membrane of the lips and tongue has a bluish-red color, the whites of the eyes seem to be filled with blood.

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

Itchy skin

Almost half of patients with polycythemia develop severe itching, especially after taking a warm bath. This phenomenon acts as a specific sign of polycythemia vera. Itching occurs due to the release of active substances into the blood, in particular histamine, which is able to expand the skin capillaries, which leads to increased blood circulation in them and the appearance of specific sensations.

Erythromelalagia

This phenomenon is characterized by short-term severe pain in the area of \u200b\u200bthe fingertips. They provoke an increase in the level of platelets in the small vessels of the hand, as a result, numerous microthrombi are formed, clogging arterioles and blocking the flow of blood to the tissues of the fingers. External signs of this condition are redness and the appearance of cyanotic spots on the skin. In order to prevent thrombosis, it is recommended to take aspirin.

Splenomegaly (enlarged spleen)

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

Duodenal and stomach ulcers

Such a serious surgical pathology develops as a result of thrombosis of small vessels of the mucous membrane of the digestive tract. The result of acute circulatory disorders is the necrosis (necrosis) of a section of the organ wall and the formation of an ulcer defect in its place. In addition, the stomach's resistance to Helicobacter (a microorganism that causes gastritis and ulcers) decreases.

Blood clots in large vessels

The veins of the lower extremities are more susceptible to this pathology. Blood clots, breaking off from the vessel wall, can, bypassing the heart, penetrate into the pulmonary circulation (lungs) and provoke PE (pulmonary embolism) - a condition incompatible with life.

Bleeding gums

Despite the fact that the number of platelets in the peripheral blood changes and its coagulability increases, gingival bleeding can occur with 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. This symptom causes damage to the arteries of the legs, their narrowing and, as a result, impaired blood circulation. This pathology is called "obliterating endarteritis"
Flat bone pain. The increased activity of the bone marrow (the place of development of blood cells) provokes the sensitivity of flat bones to mechanical stress.

Deterioration of the general condition of the body

With a disease such as polycythemia, the symptoms may be similar to signs of other pathologies (for example, anemia): headaches, constant fatigue, tinnitus, dizziness, flickering "goosebumps" before the eyes, shortness of breath, flushing of the head. An increase in the viscosity properties of blood activates the compensatory reaction of the vessels, as a result, an increase in blood pressure occurs. With this pathology, complications are often observed in the form of heart failure and microcardiosclerosis (replacement of the muscle tissue of the heart with connective tissue that fills the defect, but does not perform the necessary functions).

Diagnostics

Polycythemia is detected according to the results of a general blood test, in which it is found:

increased number of red cells from 6.5 to 7.5 10 ^ 12 / l;

increased hemoglobin level - up to 240 g / l;

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

Since the number of erythrocytes cannot be calculated based on measurements of the above values, radionuclide diagnostics are used for measurement. If the mass of red blood cells exceeds 36 ml / kg in men and 32 ml / kg in women, then this reliably indicates the presence of Vakesz disease.

In polycythemia, the morphology of erythrocytes is preserved, that is, they do not change their normal shape and size. However, when anemia develops as a result of increased bleeding or frequent bloodletting, microcytosis (a decrease in red blood cells) is observed.

Polycythemia: Treatment

Bloodletting has a good therapeutic effect. It is recommended to remove 200-300 ml of blood weekly until the ESR level drops to the desired value. If there are contraindications for bloodletting, the percentage of erythrocytes can be restored by diluting the blood by adding a liquid part to it (high molecular weight solutions are injected intravenously).

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

With such an ailment as polycythemia vera, treatment involves adherence to a certain diet. It is recommended to limit the consumption of meat and fish products, since they contain a high amount of protein, which actively stimulates the activity of the hematopoietic organs. You should also give up fatty foods. Cholesterol contributes to the development of atherosclerosis, as a result of which blood clots occur, which are already formed in large numbers in people suffering from polycythemia.

With such a disease, it is recommended to give preference to dairy and herbal products, as well as limit physical activity.

Also, if diagnosed with polycythemia, treatment may include chemotherapy. It is used for increased thrombocytosis and severe itching. As a rule, this is a "cytoreductive agent" (the drug "Hydroxycarbamide").

Until recently, injections of radioactive isotopes (usually phosphorus-32) were used to suppress bone marrow. Today, such treatment is increasingly being refused, due to the high rate of leukemic transformation.

The therapy also includes injections of interferon; in the treatment of secondary thrombocytosis, the drug "Anagrelide" is used.

With this pathology, a bone marrow transplant is very rarely performed, since polycythemia is a disease that is not fatal, provided, of course, adequate treatment and constant monitoring.

Polycythemia in newborns

Polycythemia is a pathology that can be found in newborn babies. This disease is the response of the baby's body to the transferred hypoxia, which could be provoked by placental insufficiency. The baby's body begins to synthesize a large number of red blood cells to correct hypoxia.

In addition to respiratory conditions, newborns can develop polycythemia vera. Twins are especially at risk.

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

Neonatal polycythemia has several stages of the course: initial, stage of polyiferation and exhaustion. We will briefly describe them.

The initial stage of the disease has practically no clinical manifestations. It is possible to identify polycythemia in a child at this stage only by examining the indicators of peripheral blood: hematocrit, hemoglobin and erythrocyte levels.

At the stage of polyiferation, an increase in the liver and spleen develops. Plethoric phenomena are observed: the skin acquires a characteristic "plethoric-red" shade, the child shows anxiety when touching the skin. Plethoric syndrome is complemented by thrombosis. In the analyzes, there is a change in the number of erythrocytes, platelets and leukocyte shifts. Indicators of all blood cells can also increase, this phenomenon is called "panmyelosis".

The wasting stage is characterized by significant loss of body weight, asthenia and wasting.

For a newborn, such clinical changes are extremely difficult and can provoke irreversible changes and subsequent death. Polycythemia can interfere with the production of certain types of white blood cells, which are responsible for the body's immune system. As a result, the infant develops severe bacterial infections, eventually leading to death.

After reading this article, you have learned more about such a pathology as polycythemia. We examined the symptoms and treatment in as much detail as possible. We hope you find this information useful. Take care of yourself and be healthy!

The lecture was read by: MD, prof. Pyasetskaya N.M., dept. Neonatology on the basis of the Ukrainian Children's Specialized Hospital of the Ministry of Health of Ukraine "OKHMATDET".

Polycythemia - This is a malignant increase in the number of cells of blood germs: erythroid to a greater extent, platelet and neutrophilic to a lesser extent.

ICD-10 code: R61, R61.1

Clinical diagnosis:

Neonatal polycythemia (erythrocytosis, primary polycythemia, true) is diagnosed when:

Ht veins. (Venous hematocrit)\u003e 70% or venous Hb\u003e 220 g / l.

An example of a diagnosis: Primary polycythemia with severe erythrocytosis, thrombocytosis and leukocytosis, grade II. (erythremic stage). Hepatosplenomegaly. Vascular thrombosis.

The incidence is:

2-5% - in healthy full-term newborns,

7-15% - in premature babies.

Polycythemia problem:

reduced transport function of erythrocytes;
oxygen supply to tissues is impaired (Ht veins\u003e 65%).

The causes of polycythemia:

1) Intrauterine hypoxia (increased erythropoiesis):

gestosis of pregnant women;
severe heart disease of the mother;
placental insufficiency of an infant with intrauterine hypotrophy;
postmaturity (additional fluid loss);

2) Lack of oxygen delivery (secondary polycythemia of the newborn):

impaired ventilation (lung disease);
congenital blue heart defects;
congenital methemoglobinemia;

3) Risk group for the development of neonatal polycythemia in newborns:

Diabetes mellitus in the mother;
Late clamping of the umbilical cord (\u003e 60 sec);
Feto-fetal or maternal-fetal transfusion;
Congenital hypothyroidism, thyrotoxicosis;
Down syndrome;
Wiedemann-Beckwith syndrome;

Classification of polycythemia in newborns:

1) Neonatal polycythemia:

2) Primary polycythemia:

Polycythemia vera;
Erythrocytosis (benign familial polycythemia of the newborn);

3) Secondary polycythemia - the result of insufficient oxygen delivery (promotes the synthesis of erythropoietin, which accelerates erythropoiesis and increases the number of red blood cells), or a failure in the hormone production system.

A. Oxygen deficiency:

Physiological: during intrauterine development; low oxygen content in the inhaled air (high altitude).
Pathological: impaired ventilation (lung disease, obesity); arteriovenous fistulas in the lungs; congenital heart disease with an intracardiac shunt from left to right (tetrad of Fallot, Eisenmenger complex); hemoglobinopathies: (methemoglobin (congenital or acquired); carboxyhemoglobin; sulfhemoglobin; hemoglobinopathies with a high affinity of hemoglobin for oxygen; lack of 2,3-diphosphoglycerate mutase in erythrocytes.

B. Enhanced erythropoiesis:

Endogenous causes:

a) on the part of the kidneys: Wilms tumor, hypernephroma, renal ischemia, vascular diseases of the kidneys, benign neoplasms of the kidneys (cysts, hydronephrosis);

b) from the adrenal glands: pheochromocytoma, Cushing's syndrome, congenital adrenal hyperplasia with primary aldosteronism;

c) from the liver: hepatoma, focal nodular hyperplasia;

d) from the side of the cerebellum: hemangioblastoma, hemangioma, meningioma, hepatocellular carcinoma, liver hemangioma;

e) from the side of the uterus: leiomyoma, leiomyosarcoma.

Exogenous causes:

a) the use of testosterone and related steroids;

b) the introduction of growth hormone.

4) False (relative, pseudocythemia).

Gaisbeck's syndrome - also refers to false polycythemia, since it is characterized by an increase in the level of red blood cells in the general blood test and an increase in blood pressure, which in combination gives similar clinical manifestations as polycythemia, but hepatosplenomegaly and the appearance of immature forms of leukocytes are not observed.

Stages of neonatal polycythemia:

I Art. (initial) - the clinical picture is erased, the disease is sluggish. The first stage can last up to 5 years. The disease can be suspected only with a laboratory blood test, in which moderate erythrocytosis is observed. Objective data are also not very informative. The spleen and liver are slightly enlarged, but this is not a pathognomonic sign of this disease. Complications from internal organs or blood vessels are extremely rare.

II Art. (proliferation) - the clinic is characterized by the height of the disease. There is a pletora, hepatosplenomegaly, depletion of the body, manifestation of thrombosis, convulsions, tremor, dyspnea. In the general analysis of blood - erythrocytosis, thrombocytosis, neutrophillosis with a shift to the left, or panmyelosis (an increase in the amount of all blood elements). In the blood serum, the content of uric acid rises (normal \u003d up to 12 years - 119-327 μmol / l), which is synthesized in the liver and excreted by the kidneys. It circulates in blood plasma in the form of sodium salts.

III (emaciation, anemic) - clinical signs in the form of pletora, hepatosplenomegaly, general weakness, significant weight loss. At this stage, the disease becomes chronic and myelosclerosis may occur.

Syndromes that are accompanied by an increased level of Ht veins.

Blood hyperviscosity (not synonymous with polycythemia) is the result of increased levels of fibrinogen, IgM, osmolarity and blood lipids. Addiction with polycythemia becomes exponential when Htven exceeds 65%.
Hemoconcentration (relative polycythemia) is an increased level of hemoglobin and hematocrit due to a decrease in plasma volume due to acute dehydration of the body (exicosis).

General Clinic for Polycythemia:

Pletora (with primary polycethemia) is a general plethora of the body. There is a reddening of the face (becomes purple), a strong, high pulse, "beating in the temples", dizziness.
Insufficient filling of capillaries (acrocyanosis).
Dyspnea, tachypnea.
Depression, drowsiness.
Weakness in sucking.
Persistent tremor, muscle hypotonia.
Convulsions.
Bloating.

Complications (clinical conditions associated with polycythemia and hemoconcentration syndrome (thickening) of the blood):

Pulmonary hypertension with the development of PFC syndrome (persistent fetal circulation).
Increased systemic blood pressure.
Venous congestion in the lungs.
Increased tension on the myocardium.
Hypoxemia.
Metabolic disorders (hyperbilirubinemia, hypocalcemia, hypomagnesemia).
Increased glucose utilization (hypoglycemia)
Hepatomegaly.
Intracranial hemorrhage, convulsions, apnea.
Renal vein thrombosis, acute renal failure (acute renal failure), oliguria.
Ulcerative necrotizing enterocolitis.
Decreased blood circulation in the gastrointestinal tract, kidneys, brain, myocardium.

Diagnostics.

Laboratory data:

Ht veins
general blood analysis

It should be remembered that 4-6 hours (sometimes earlier) after birth, hemoconcentration necessarily occurs (rise in hematocrit, hemoglobin, leukocytes) due to certain physiological mechanisms.

Additional examinations:

platelets (thrombocytopenia),
blood gases
blood sugar (hypoglycemia),
bilirubin (hyperbilirubinemia),
urea,
electrolytes,
x-ray of the lungs (with RDS).

If necessary (determination of blood hyperviscosity), determine fibrinogen, IgM, blood lipids, calculate the osmolarity of the blood.

Differential diagnosis of true neonatal polycythemia, true secondary polycythemia due to hypoxia and pseudo-polycythemia (relative).

True neonatal polycythemia:

There is granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of erythrocytes is increased;
The regulator of erythropoiesis (erythropoietin) is normal or decreased;

True secondary polycythemia due to hypoxia:

The mass of erythrocytes is increased;
Plasma volume unchanged or reduced;
The regulator of erythropoiesis (erythropoietin) is increased;
Decreased or normal arterial oxygen saturation.

Pseudo-polycythemia:

No granulocytosis, thrombocythemia, hepatosplenomegaly;
The mass of red blood cells is unchanged;
Plasma volume is reduced;
The erythropoiesis regulator (erythropoietin) is normal;
Normal arterial oxygen saturation.

Treatment of polycythemia.

1) General activities:

Ht vein level control:

a) with Ht veins 60-70% + no clinical signs \u003d control after 4 hours

b) with Ht veins\u003e 65% + clinical signs \u003d normovolemic hemodilution or partial exchange transfusion (exfusion).

Repeated control of Ht veins: 1, 4, 24 hours after hemodilution or partial exchange transfusion

Normovolemic hemodilution:

Purpose: to reduce the level of Ht veins to 50-55% by diluting the blood (more often this method is used in the presence of dehydration).

Partial exchange transfusion:

Formula for calculating the required volume (ml) of exfusion - infusion or hemodilution:

V (ml) \u003d BCC child (ml / kg) * (Ht child - Ht desired) / Ht child, where

V (ml) - volume of partial exchange transfusion (infusion)

Ht desired ≈ 55%

BCC of a full-term baby - 85-90 ml / kg

BCC of a premature baby - 95-100 ml / kg

Example:

Child's HT - 71%;

Ht desired - 55%;

BCC of a child - 100 ml / kg;

Child's body weight - 3 kg

V (ml) \u003d 100 x 3 x (71% - 55%) 300 ml x 16% / 71% \u003d 67.6 ml. or 17 ml. x 4 times *

* Note:Do not use the pendulum technique. This technique increases the risk of developing necrotizing enterocolitis. It is necessary to carry out simultaneously in equal volumes of exfusion - transfusion using different vessels.

Solutions that can be used for hemodilution and partial exchange transfusion:

physiological solution (0.85% sodium chloride solution);
ringer's solution or Ringer's lactate;
colloidal solutions based on hydroxyethyl starch (HES) - 6%, 10% Refortan solution (indications for the use of this solution are hemodilution, correction of hemodynamic disturbances, improvement of rheological properties of blood, and others). There is little experience in neonatology.

Human plasma (FFP) should not be used.

Forecast.

With latent (asymptomatic) polycythemia, the risk of neurological disorders increases.

THE EPIDEMIC OF INSTANT CUT CUTTING:
=================
Physiologically, what happens to a newborn at birth? Global restructuring and adaptation. Up to this point, throughout his life in the womb, the baby's circulatory system included blood from his own body, blood from the umbilical cord, and blood pumped by the placenta. All three are part of one indivisible system. In the mother's womb, the baby received all nutrition from the placenta, she also removed all decay products. Immediately after birth, the child must rebuild his entire blood circulation system so that all vital systems, including the lungs, liver, kidneys, digestive system and other organs, which until that moment were in a certain “dormant” state, are included in the work.

Is it important or not to leave the umbilical cord intact, not to cut it immediately after delivery?

Extremely important. Imagine a finger that is heavily and for a long time, where no blood has flowed for several minutes. After a certain time, the finger will turn purple, if not white. By releasing it, we will allow the blood to return to the transferred part. But the stronger and longer we squeeze the blood flow in the finger, the slower it will return to this bloodless part. In much the same way, the blood balance of the newborn after childbirth is replenished. This process takes time.

Approximately 66 ml of blood passes from the baby to the placenta at the moment of maximum compression during passage through the birth canal. The return of these 66 ml is vital for the baby at birth.
According to recent studies, it has been established that a newborn receives 80% of the blood due to him during the first 30-40 seconds of life. And this is very good news for babies who will be destined to be born in a family home. But what about the remaining 20% \u200b\u200bof his rightfully owned blood? How about those newborns who, for one reason or another, take slightly longer than the average newborn to replenish their full blood volume? After all, we are all very different, with a very individual physiology.

Studies by the World Health Organization (WHO - World Health Organization) show that when the umbilical cord is cut off immediately, a newborn loses an average of 100-150 ml of blood. Expensive! This is 25 - 45% of the total blood volume of a newborn !!!
The lungs of an unborn baby are filled with fluid. Hundreds of capillaries surrounding the alveoli, the air sacs of the lungs, throughout pregnancy are in a compression, that is, compressed state, passing only 10% of the blood flow to the lungs. At the time of birth, these blood vessels must be filled with blood so that the fluid filling the lungs can escape from there into the lymph flow and circulatory system.

Throughout the entire pregnancy, the placenta provided all the baby's life activity: it fed, it also removed the decay products. At the time of childbirth, the liver, kidneys, the entire digestive system and many other organs will have to be activated and included in 100% work. Why do they need extra blood too! Where can a baby get this blood when the umbilical cord is cut immediately? Not only can the vital organs not receive the necessary and due volume of blood, but also the brain. The epidemic of immediate cutting of the umbilical cord is associated with the epidemic of autism: the lack of adequate blood supply to the tissues of the brain. It is not necessary to go that far, but the fact is, and the trouble is, we never know how serious the consequences of an instantaneous cutting of the umbilical cord can really be. Nobody dies in such a situation. The human body is very clearly programmed for survival. The center will draw the required blood from the periphery. A newborn who has an immediate cut of the umbilical cord and has lost about 100 ml of blood experiences the shock of blood loss, which is equivalent to the loss of 1000-15000 ml of blood to an adult. Everyone knows that such blood loss requires transfusion. In family homes and hospitals, instantaneous cutting of the umbilical cord is a standard procedure and the shock experienced by newborns is commonplace. Does every mother want a similar beginning in life for her child?

This 100ml of blood, rightfully owed to a child, is colossally rich in nutrients and minerals. These 100 ml of blood contain approximately 30 mg of iron. This amount is found in about 100 liters of breast milk! It is not difficult to understand that a baby who is bleeding at birth will be at a greater risk of anemia, which can be felt over the next 6 years. It is known that neonatal anemia has a direct impact on impaired brain development. Anemia is a lack of oxygen in the body. This is perhaps the strongest argument in favor of delayed cord cutting in medical circles to date.

With the receipt of these 100 ml of blood, the newborn receives 30-40% more red cells - erythrocytes, and with them hemoglobin, the carrier of the oxygen molecule.

These 100 ml of blood are also rich in the protein albumin, which creates osmotic pressure in cells, helping the newborn to remove fluid from the lungs in the shortest possible time, and thus adapt to the requirements of our atmospheric world with minimal effort and discomfort.

A newborn whose umbilical cord was cut off instantly at birth will not receive the volume of stem cells due to him. Stem cells are special cells, they migrate into the child's bone tissue and participate in the formation of all necessary cells in the body. For example, when lung cancer occurs, the body needs cells of the lung tissue. In such a situation, previously undifferentiated stem cells take on a similar responsibility and highly specialized work.

Natural placental blood transfusion is especially important for newborns who need help establishing a breathing cycle. Because even after being born, the baby still receives oxygen from the placenta. Therefore, in critical situations, when the newborn needs emergency assistance in breathing, the umbilical cord should definitely not be cut until the newborn adapts to the new requirements of our atmospheric world.

The most common myth associated with delayed blood transfusion is the “risk of developing polycythemia of the newborn”. Polycythemia is an increase in the number of red blood cells in a unit of blood. What happens to the millions of newborns whose umbilical cord has not been pinched or cut at all? This is what happens. A newborn, indeed, receives 150% more red cells, the volume of which is so confusing for the entire medical world. But let's exhale and sort it out calmly. The fact is that newborns need all this volume of red cells, which carry a hemoglobin molecule with them. The excess amount of red cells in the next day ends its life, separating hemoglobin, which in the process of metabolism forms a substance - biliverdin. Biliverdin, in turn, under the action of liver enzymes is converted into bilirubin - a yellow substance, a breakdown product of hemoglobin.

Bilirubin is the only antioxidant in the newborn's body. Only! Almost all healthy newborns have a mild form of jaundice in the first days of life. Don't worry. This is a completely physiological jaundice. This is not a pathology! Why do newborns need this bilirubin in such an excess amount? That's what it is for. The level of oxygen concentration in the blood of a newborn until the moment of delivery is significantly lower than the oxygen concentration in our atmospheric air. When a newborn is born, a newborn inhales a "dose" of oxygen, which is unusual for his system, which leads to inevitable oxidative processes (and those, in turn, we all know, to the formation of free radicals!). So the concentration of bilirubin (taken from a high volume of erythrocytes obtained from placental transfusion) is the only compensatory mechanism of the newborn in oxidative processes that can lead to serious disorders and toxicity of the system as a whole. Having completed its task and coping with oxidative processes, bilirubin is easily excreted from the system of the newborn in the urine. So polycythemia is not a consequence of delayed cutting of the umbilical cord. Polycythemia is the first and very alarming signal to pay very close attention to the condition of the newborn. The fact is that polycythemia in the most frequent cases is a sign of liver dysfunction, endocrine disorders of blood diseases, or other serious congenital defects.

Well, there is another myth associated with the fact that "all the blood from the newborn will pour into the placenta if it is below the newborn." It is worth noting here that the newborn and the placenta are not two troughs connected by a hose. Feto-placental transfusion takes place in a very delicate manner. After each contraction, the placenta "gives" the newborn a certain volume of blood, to which the system of the second responds not immediately, but after a certain pause. If the volume exceeds the requirements of the newborn's body, it sends the excess back to the placenta. Sometimes too much, and therefore the baby must "wait" for the return of the missing blood volume. After the next contraction, the placenta repeats the transfusion, and if the volume again exceeds the requirements of the newborn's system, he "pours" the excess back to the placenta. This "calibration" occurs in some cases within one minute, in others within 15 minutes, and sometimes longer. Therefore, until the pulsation of the umbilical cord stops, it should in no case be squeezed. After all, we do not know at which stage of the transfusion we cut the umbilical cord. Concerned so much about polycythemia, the umbilical cord may be cut at the very moment when too much blood has been donated by the placenta to the newborn.

An excerpt from the audio recording "The first hour of a child's life"
Family Doctor Sarah Buckley, Australia (mother of four home-born children)
Midwife Gail Hart, USA (over 40 years at home)
Obstetrician Gynecologist John Stevenson, Australia (over 40 years of hospital practice, about 10 years of home delivery, during which Dr. John delivered 1239 women)
Obstetrician-Gynecologist Michel Odent, France (one of the leading lawyers in the world of natural childbirth)