Polycythemia vera prognosis. Polycythemia vera: causes, symptoms, diagnosis, treatment. Reasons for the development of the disease

Polycythemia (synonymous with Wakez's disease) is a chronic disease of the hematopoietic system, characterized by a persistent increase in the amount of total blood volume, and increased production in the bone marrow of not only erythrocytes, but also leukocytes and.

Polycythemia belongs to the group of leukemias. Pathological anatomy reveals a sharp plethora of internal organs, often vascular thrombi, heart attacks, hemorrhages. In the bone marrow, the phenomena of hyperplasia (an increase in cellular elements) of an erythroblastic germ, in the diaphysis of long bones - the transformation of fatty bone marrow into red.

Polycythemia develops gradually and has a progressive course. Clinically manifested by dark red skin with a cyanotic tint, plethora of mucous membranes with possible bleeding from the gums, stomach, intestines, uterus, enlarged spleen and liver, hypertension. In the blood, the content of erythrocytes (6,000,000-10,000,000), hemoglobin (20-23 g%) is increased, it is slowed down to 1 mm in 1 or even 2 hours.

The course of the process is long, the prognosis worsens if the vessels of vital organs develop.

Treatment - in a hospital with repeated bloodletting, cytostatic drugs (myelosan, imifos, myelobromol).

Polycythemia red, true (polycythaemia, rubra, vera; from Greek poly - many, kytos - cell and haima - blood; synonym: erythremia, Wakez's disease) - a chronic disease of the hematopoietic apparatus of unknown etiology, characterized by a persistent increase in the number of red blood cells and total blood volume with the expansion of the bloodstream, an increase in the spleen and increased activity of the bone marrow, and the hyperplastic process concerns not only erythropoiesis, but also leuko- and thrombocytopoiesis.

Recently, the neoplastic theory of pathogenesis has been established. Polycythemia is considered an independent disease and belongs to the group of myeloproliferative leukemias, considering it as chronic erythromyelosis (see) with a predominant increase in the function of erythropoiesis.

Pathological anatomy reveals a sharp plethora of internal organs, often vascular thrombi, heart attacks, hemorrhages. The spleen is enlarged, hard, dark blue-red. The liver is often enlarged and may be cirrhotic. In the diaphysis of tubular bones - the transformation of fatty bone marrow into red. Hyperplasia of an erythroblastic germ in the bone marrow and in extramedullary foci of hematopoiesis retains the usual type of regeneration, hyperplasia of myeloid tissue sometimes becomes similar to leukemic. Significant hyperplasia of the megakaryocytic apparatus. These changes are also detected in the clinic with sternal puncture and more clearly with trepanobiopsy of the ilium.

Clinical course and symptoms. Polycythemia develops most often in old age (40-60 years), however, cases of the disease in young and even childhood have been described. The disease usually develops gradually. The life expectancy of patients from the moment of detection of the disease now reaches an average of 13.3 years [Lawrence (J. N. Lawrence)], and in some cases even up to 30 years or more (E. D. Oak and M. A. Yasinovsky).

A special color of the integument (erythrosis) is typical: intense dark red with a cherry tint, skin color, especially pronounced on the face and distal parts of the limbs; mucous membranes are bright red, often cyanotic; the injection of scleral vessels is noticeable, the gums are loosened, often bleed, periodontal disease is detected. Plethora with an increase in the mass of circulating blood by 2-4 times, with an increase in its viscosity significantly affects the state of the cardiovascular system and blood circulation, the blood flow rate decreases by 2-3 times or more. Hypertension is one of the most important and frequent symptoms of polycythemia. It is impossible to exclude the combination of polycythemia with hypertension. Of great importance are lesions of peripheral vessels with the development of obliterating thromboangiitis, and sometimes blockage of arteries with gangrene, thrombosis of cerebral vessels, coronary arteries, splenic and renal arteries with the formation of heart attacks, thrombosis of the portal vein and its branches. There are bleeding from the nose, gums, stomach, intestines, uterus, etc., hemorrhages in the brain, abdominal cavity, spleen.

Violations of the nervous system occur from the very beginning of the disease. According to the totality of neurological symptoms, separate syndromes can be distinguished: cerebrovascular insufficiency, neurasthenic, diencephalic, vegetative-vascular, polyneuritic and erythromelalgia.

Splenomegaly is observed in 2/3-3/4 of all cases. Enlargement and thickening of the liver is noted in 1/3-1/2 patients.

There are no marked changes in the condition of the kidneys.

The number of erythrocytes in 1 ml3 of blood is usually 6-10 million, in some cases - 12 million. The percentage of reticulocytes is relatively low. The content of hemoglobin reaches 120-140% (20-23 g%), rarely higher. The color index is below one. The number of leukocytes is increased (in more than 1/2 of patients) and sometimes reaches 20,000-25,000 or more per 1 mm 3, mainly due to neutrophils with a shift to the left to metamyelocytes and myelocytes. The greatest number of leukocytes and the appearance of younger forms is observed with the development of myeloid leukemia. The number of platelets also increases for the most part - up to 600,000 and even sometimes up to 1 million or more in 1 mm 3. ROE is slowed down to 1 mm for 1 and even 2 hours. The ratio between the mass volume of erythrocytes and plasma, determined using hematocrit, increases to 85:15. Quite frequent are pains in the bones with a change in the structure of their tissue, especially in the epimetaphyses of long tubular bones.

In the early stages, the appearance of neurovascular disorders acquires diagnostic value. With a pronounced picture of polycythemia, recognition is primarily based on the classic triad: erythrosis, polyglobulia, splenomegaly. Polycythemia should be distinguished from a number of conditions that are also characterized by an increase in the number of red blood cells per unit volume of blood - the so-called polyglobulia, or erythrocytosis. False polyglobulia is not associated with a real increase in the number of red blood cells in the peripheral blood, but occurs due to thickening of the blood, for example, with significant diarrhea and vomiting (for example, with cholera), increased sweating, and profuse diuresis. Symptomatic polyglobulia can be relative when the number of erythrocytes in the peripheral blood increases, mainly due to their redistribution (when deposited blood is released), for example, during rapid ascent to altitude, acute heart and lung failure.

True absolute polyglobulia with a reactive increase in bone marrow erythropoiesis is of particular importance in differential diagnosis. Most often, it is associated with a prolonged anoxic state: in residents of highlands, with congenital heart defects, acquired defects with severe circulatory failure, sclerosis of the branches of the pulmonary artery, pneumosclerosis, pronounced emphysema and other lung diseases. This includes polyglobulia when exposed to toxic substances on hematopoiesis. They become important in the occurrence of polyglobulia and damage to the central nervous system (for example, the subthalamic region) by an inflammatory or neoplastic process, some endocrine disorders (Itsenko-Cushing's syndrome), etc. In the differential diagnosis between polycythemia and polyglobulia, an increase in the spleen, leukocytosis with a neutrophilic shift to the left indicate in favor of polycythemia , thrombocytosis, a significant increase in the total mass of blood and especially erythrocytes with a high hematocrit, trephine biopsy data, a significant increase in the activity of neutrophil alkaline phosphatase, a high rate of Fe69 absorption from plasma, etc.

The prognosis, given the progressive nature of the course of the disease, the absence of spontaneous remissions and spontaneous cure, is generally unfavorable, although with modern therapy, life and working capacity are preserved longer. The cause of death is most often vascular complications - thrombosis, hemorrhage, bleeding, circulatory failure or transition to the picture of myelosis or, more rarely, to hemocytoblastosis, to aplastic anemia due to the development of myelofibrosis and osteomyelosclerosis.

Treatment is pathogenetic. Bloodletting (usually 400-500 ml repeatedly at intervals of 2-3-5 days until a clear decrease in red blood values) is especially indicated for high blood pressure, the threat of cerebral complications and high hematocrit. This method gives relief only for the next few months, and is often used in combination with radiophosphorus therapy.

Radiation therapy is the most effective. It is more expedient to irradiate the entire body with X-rays.

In recent years, widely used radioactive phosphorus (P 32), which is administered on an empty stomach through the mouth in the form of NaHP 32 O 4 in 20-40 ml of 40% glucose solution, can also be used intravenously. Contraindications to the use of P 32 - liver disease with significant dysfunction, kidney disease, leukopenia (below 4000 in 1 mm 3), thrombocytopenia (below 150,000 in 1 mm 3).

Fractional administration of P 32 is more widespread (to receive 1.5 - 2 microcuries once every 4-7-10 days, in total for a course of 6-8 microcuries in accordance with the indicators of red blood and the weight of the patient). Prior to treatment with P 32, it is recommended to carry out 2-3 phlebotomies of 400-500 ml at intervals of 2-3 days, especially in patients with severe symptoms of cerebrovascular accident, the number of red blood cells above 7.5-8 million in 1 mm 3 and high indicators of hematocrit (65-70).

The clinical effect is felt already after 2-4 weeks, and hematological remission occurs after 2-4 months. after the start of treatment and usually lasts 2-3 years or more.

In the treatment of P 32, complications in the form of leukopenia, thrombocytopenia, and less often anemia, which are transient, can be observed.

Repeated courses of treatment with P 32 are prescribed for relapses of the disease.

Hepatosplenomegaly may also develop. The diagnosis is established on the basis of a complete blood count, testing for the presence of 1AK2 gene mutations, and clinical criteria. Treatment includes the use of low-dose aspirin in all patients and myelosuppressive drugs in high-risk patients. Bloodletting used to be the standard of care, but its role is now controversial.

What is polycythemia vera

Polycythemia vera is the most common myeloproliferative disorder. Its incidence in the US is 1.9/100,000, with the risk increasing with age. PI is somewhat more common in men. PI is very rare in children.

Pathophysiology of polycythemia vera

With IP, increased proliferation of all cell sprouts is noted. In this regard, PV is sometimes called panmyelosis due to an increase in the content of representatives of all 3 peripheral blood cell lines. Increased production of a single red blood cell is called erythrocytosis. Isolated thrombocytosis can occur with PV, but more often it occurs for other reasons (secondary erythrocytosis).

Extramedullary hematopoiesis can occur in the spleen, liver, and other organs that can serve as a site for the formation of blood cells. The turnover of peripheral blood cells increases. Ultimately, the disease may enter a debilitating phase, the manifestations of which are indistinguishable from primary myelofibrosis. Transformation to acute leukemia is rare, but the risk increases with the use of alkylating agents and radioactive phosphorus. The latter should only be used on rare occasions or not at all.

Complications. With IP, the volume of circulating blood increases and its viscosity increases. Patients are prone to developing thrombosis. Thrombosis can occur in most vessels, leading to strokes, transient ischemic attacks, or Budd-Chiari syndrome. In the past, experts believed that increased blood viscosity was a risk factor for thrombosis. Recent studies indicate that the risk of thrombosis may primarily depend on the severity of leukocytosis. However, this hypothesis remains to be tested in prospective studies specially designed for this purpose.

Platelet function may be impaired, which increases the risk of bleeding. Accelerated cell turnover can cause an increase in the concentration of uric acid, thereby increasing the risk of developing gout and the formation of kidney stones.

genetic factors. Clonal hematopoiesis is a hallmark of PV. This indicates that the cause of proliferation is a mutation in hematopoietic stem cells. The JAK2 V617F mutation (or one of several other rarer JAK2 gene mutations) is found in virtually all patients with PV. However, it can be said with almost complete certainty that there are other mutations that underlie the disease. They keep JAK2 protein in a state of constant activity, which leads to excessive cell proliferation regardless of the concentration of erythropoietin.

Signs and symptoms of polycythemia vera

It is detected either by chance by high hemoglobin or by symptoms of increased viscosity, such as fatigue, loss of concentration, headaches, dizziness, blackouts, itchy skin, nosebleeds. Sometimes it is manifested by diseases of the peripheral arteries or damage to the vessels of the brain. Patients are often plethoric, and most have an enlarged spleen. Thrombosis and often peptic ulceration may occur, sometimes complicated by bleeding.

Polycythemia vera is often asymptomatic. Sometimes an increase in the number of circulating red blood cells and an increase in viscosity are accompanied by weakness, dizziness, visual disturbances, fatigue and shortness of breath. Itching is a common symptom, especially after a shower. There may be redness of the face and dilated retinal veins, as well as redness and soreness of the palms and soles, sometimes in combination with ischemia of the fingers (erythromelalgia). Hepatomegaly is often observed, splenomegaly (sometimes pronounced) occurs in 75% of patients.

Thrombosis may cause symptoms in the affected area (eg, neurologic deficits in stroke or transient ischemic attacks, leg pain, leg swelling, or both in lower extremity vascular thrombosis, unilateral vision loss in retinal vascular thrombosis).

Bleeding occurs in 10% of patients.

An accelerated metabolism can cause low-grade fever and lead to weight loss, which indicates the transition of the disease to the phase of exhaustion. The latter is clinically indistinguishable from primary myelofibrosis.

Diagnosis of true polycythemia

General blood analysis.
Testing for mutations in the JAK2 gene.
In some cases, a study of the bone marrow and the determination of the plasma concentration of erythropoietin.
Application of WHO criteria.

PV is often suspected at the stage of a complete blood count, but it should also arise in the presence of relevant symptoms, in particular Budd-Chiari syndrome (it is worth noting, however, that in some patients Budd-Chiari syndrome develops before an increase in hematocrit). Neutrophilic leukocytosis and thrombocytosis are common but not mandatory manifestations. Patients with an isolated increase in hemoglobin or erythrocytosis may also have PV, but in such cases, secondary erythrocytosis should be ruled out first. PI can also be suspected in some patients with normal hemoglobin levels but microcytosis and signs of iron deficiency. This combination of features may occur with hematopoiesis occurring in the presence of limited iron stores, which is a hallmark of some cases of PV.

WHO has developed new diagnostic criteria. Thus, patients with suspected PV should usually be tested for JAK2 mutations.

Examination of a bone marrow sample is not always necessary.

In cases where it is performed, panmyelosis, large size and crowding of megakaryocytes usually attract attention in the bone marrow. In some cases, reticulin fibers are found. However, no changes in the bone marrow can distinguish PV with absolute certainty from other pathological conditions (for example, congenital familial polycythemia) accompanied by erythrocytosis.

Plasma erythropoietin concentrations in patients with PV are usually low or in the lower limit of normal. An increased concentration indicates the secondary nature of erythrocytosis.

In some cases, testing for endogenous colony formation of erythroid cells in vitro is performed (erythrocyte precursors taken from the peripheral blood or bone marrow of patients with PV, unlike those in healthy people, can form erythroid cells in culture without the addition of erythropoietin).

Determination of total RBC mass using chromium-labeled RBCs can help distinguish polycythemia vera and relative polycythemia, as well as distinguish polycythemia from myeloproliferative disorders. However, the technique for performing this test is complex. It is not normally carried out given its limited availability and the fact that it is standardized for use at sea level only.

Non-specific laboratory abnormalities that may occur with PV include increased vitamin B12 concentration and increased B12 binding capacity, as well as hyperuricemia and hyperuricosuria (present in >30% of patients), increased expression of the PRV-1 gene in leukocytes, decreased expression of the C-mpl (thrombopoetin receptor) gene in megakaryocytes and platelets. These tests are not required to establish a diagnosis.

Diagnosis of polycythemia is discussed in the subsection "Elevated hemoglobin". For diagnosis, an increase in the mass of erythrocytes in the absence of reasons for secondary erythrocytosis and splenomegaly is important. The number of neutrophils and platelets is often increased, an abnormal karyotype can be found in the bone marrow, and in vitro culture of the bone marrow shows autonomous growth in the absence of the addition of growth factors.

Polycythemia vera prognosis

In general, PI is associated with shortened life expectancy. The median survival of all patients is 8 to 15 years, although many live much longer. A common cause of death is thrombosis. Complications of myelofibrosis and the development of leukemia are next in frequency.

The average survival after diagnosis in patients receiving treatment exceeds 10 years. Some patients live more than 20 years; however, cerebrovascular and coronary complications occur in 60% of patients. The disease may progress to another myeloproliferative disorder; myelofibrosis develops in 15% of patients. Acute leukemia appears mainly in patients treated with radioactive phosphorus.

Treatment of true polycythemia

aspirin treatment,
Possible bleeding
Possible myelosuppressive therapy.

Therapy should be selected individually, taking into account age, gender, health status, clinical manifestations and the results of hematological studies. Patients are divided into high risk group and low risk group. Patients >60 years of age with a history of thrombosis or transient ischemic attacks, or both, are considered high-risk.

Aspirin. Aspirin reduces the risk of thrombosis. Therefore, patients undergoing only bloodletting or phlebotomy should receive aspirin. Higher doses of aspirin are associated with an unacceptably high risk of bleeding.

Bloodletting. Bloodletting has been the mainstay of treatment for both high- and low-risk patients, as experts believed it reduced the likelihood of thrombosis. The validity of phlebotomy is currently controversial, as new research indicates that hemoglobin levels may not be correlated with risk of thrombosis. Some clinicians no longer adhere to strict bloodletting recommendations. Bloodletting is still one of the possible alternatives for any patient. In a minority of patients with flushed skin and increased blood viscosity, phlebotomy may improve symptoms. The standard hematocrit threshold above which bloodletting is performed is >45% in men and >42% in women. As soon as the hematocrit value falls below the threshold, it is checked monthly and maintained at the same level by additional phlebotomies, which are performed as needed. If necessary, intravascular volume is replenished with crystalloid or colloid solutions.

Myelosuppressive therapy is indicated for high-risk patients.

Radioactive phosphorus (32P) has long been used to treat PV. The effectiveness of treatment is from 80 to 90%. Radioactive phosphorus is well tolerated and requires fewer clinic visits after disease control is achieved. However, the use of radioactive phosphorus is associated with an increased risk of developing acute leukemia. Leukemia that occurs after such therapy is often resistant to induction therapy and is always incurable. Thus, the use of radioactive phosphorus requires careful selection of patients (for example, the drug should be prescribed only to those patients whose life expectancy due to comorbidities does not exceed 5 years). It is worth appointing only in rare cases. Many doctors do not use it at all.

Hydroxyurea inhibits the enzyme ribonucleoside diphosphate reductase. It is also used to suppress bone marrow activity. There is no unequivocal data on the ability of hydroxyurea to provoke leukemia. However, the likelihood of transformation into leukemia exists, although it is small. Patients have weekly blood tests. After reaching an equilibrium state, the intervals between blood tests are increased to 2 weeks, and then to 4 weeks. If the level of leukocytes falls<4000/мкл или уровень тромбоцитов падает <100 000/мкл, лечение приостанавливают, а когда упомянутые показатели приходят в норму, возобновляют в дозе на 50% меньше исходной. Дозу гидроксимочевины рационально титровать до достижения практически нормальной величины гематокрита, однако данные в пользу такого титрования отсутствуют. Нормализация уровня лейкоцитов, вероятно, более важна, но как и в предыдущем случае, эта гипотеза не была подтверждена проспективными исследованиями. Подтверждения тому, что нормализация уровня тромбоцитов необходима, нет, и некоторые врачи не увеличивают дозу гидроксимочевины до тех пор, пока число тромбоцитов остается <1,5 млн/мкл. Острая токсичность - нередкое явление. В некоторых случаях у пациентов возникает сыпь, лихорадка, изменения внешнего вида ногтей, кожные язвы.

Interferon alpha-2b is used in cases where hydroxyurea fails to maintain the desired level of blood cells or when the latter is ineffective. It is worth noting that pegylated interferon alfa-2b is generally well tolerated. This drug acts on the disease at the molecular level and has a relatively low toxicity.

Alkylating drugs can provoke the development of leukemia, so they should be avoided.

Several inhibitors of the JAK2 cascade are currently in the clinical development phase. They are mainly studied in patients with advanced stages of myelofibrosis.

Treatment of complications. Hyperuricemia is corrected with allopurinol if the increase in uric acid concentration is accompanied by symptoms or if patients are receiving concomitant myelosuppressive therapy. Itching can be tried to be controlled with antihistamines, but this can sometimes be difficult to achieve. Myelosuppression is often the most effective method. An example of a potentially effective therapy would be cholestyramine, cyproheptadine, cimetedine, or paroxetine.

Bloodletting quickly relieves the symptoms of increased viscosity. 400-500 ml of blood is removed - and venesection is repeated every 5-7 days until the hematocrit drops by 45%, removing 400-500 ml of blood during each procedure (less if the patient is elderly). Less frequent but regular bloodletting maintains this level until hemoglobin becomes low due to iron deficiency. The underlying myeloproliferation is suppressed with hydroxycarbamide or interferon. Treatment with radioactive phosphorus (5 mCi 32R intravenously) is reserved for elderly patients, as it increases the risk of transformation into acute leukemia by 6-10 times. Treatment of bone marrow proliferation can reduce the risk of vascular occlusion, control spleen size, and reduce transformation to myelofibrosis. Aspirin reduces the risk of thrombosis.

The overproduction of red blood cells is most dramatic in polycythemia, but the production of white blood cells and platelets is also increased in most cases.

Polycythemia vera is a rare, chronic disease involving an overproduction of blood cells in the bone marrow (myeloproliferation).

The overproduction of red blood cells is the most dramatic, but the production of white blood cells and platelets is also increased in most cases. The overproduction of red blood cells in the bone marrow leads to an abnormally high number of circulating red blood cells. Consequently, the blood thickens and increases in volume; this condition is called hyperviscosity.

Polycythemia is an increase in the number of red blood cells in the blood. With polycythemia, hemoglobin levels and the number of red blood cells (erythrocytes) are increased, and hematocrit (the percentage of red blood cells to blood plasma) is also increased. An elevated RBC count can easily be detected with a complete blood count. A hemoglobin level greater than 16.5 g/dl in women and greater than 18.5 g/dl in men indicates polycythemia. In terms of hematocrit, values above 48 in women and above 52 in men are indicative of polycythemia.

The production of red blood cells (erythropoiesis) occurs in the bone marrow and is regulated by a series of specific steps. One of the important enzymes that regulate this process is erythropoietin. Most erythropoietin is produced in the kidneys, and a smaller part is produced in the liver.

Polycythemia can be caused by internal problems in the production of red blood cells. It's called primary polycythemia. If polycythemia is due to another underlying medical problem, it is called secondary polycythemia. In most cases, polycythemia is secondary and due to another disease. Primary polycythemia is relatively rare. Approximately 1-5% of newborns can be diagnosed with polycythemia (neonatal polycythemia).

Primary causes of polycythemia

Polycythemia vera is associated with a genetic mutation in the JAK2 gene that increases the sensitivity of bone marrow cells to erythropoietin. As a result, the production of red blood cells increases. Levels of other types of blood cells (leukocytes and platelets) are also often elevated in this condition.

Primary familial and congenital polycythemia is a condition associated with a mutation in the Epor gene that causes an increase in red blood cell production in response to erythropoietin.

Secondary causes of polycythemia

Secondary polycythemia develops due to high levels of circulating erythropoietin. The main reasons for an increase in erythropoietin are: chronic hypoxia (low oxygen levels in the blood for a long period of time), poor oxygen delivery due to the abnormal structure of red blood cells and tumors.

Some of the common conditions that can lead to an increase in erythropoietin due to chronic hypoxia or poor oxygen supply include: 1) chronic obstructive pulmonary disease (COPD, emphysema, chronic bronchitis); 2) pulmonary hypertension; 3) hypoventilation syndrome; 4) congestive heart failure; 5) obstructive sleep apnea; 6) poor blood flow to the kidneys; 7) life at high altitudes.

2,3-BPG is a deficient condition in which the hemoglobin molecule in red blood cells has an abnormal structure. In this state, hemoglobin has a higher affinity for oxygen uptake and releases less of it to body tissues. This leads to more production of red blood cells, since the body perceives this anomaly as an insufficient level of oxygen. The result is a greater number of circulating red blood cells.

Some tumors cause excessive secretion of erythropoietin, resulting in polycythemia. Common erythropoietin-releasing tumors: liver cancer (hepatocellular carcinoma), kidney cancer (renal cell carcinoma), adrenal adenoma or adenocarcinoma, uterine cancer. Benign conditions such as kidney cysts and renal obstruction can also lead to an increase in erythropoietin secretion.

Chronic exposure to carbon monoxide can lead to polycythemia. Hemoglobin has a higher affinity for carbon monoxide than for oxygen. Therefore, when carbon monoxide molecules attach to hemoglobin, polycythemia can occur to compensate for poor oxygen delivery to existing hemoglobin molecules. A similar scenario can also occur with carbon dioxide due to long-term smoking.

The cause of neonatal polycythemia (neonatal polycythemia) is often the transfer of maternal blood from the placenta or a blood transfusion. Prolonged poor oxygen delivery to the fetus (intrauterine hypoxia) due to placental insufficiency can also lead to neonatal polycythemia.

Relative polycythemia

Relative polycythemia describes conditions in which the volume of red blood cells is high due to an increased concentration of red blood cells in the blood as a result of dehydration. In these situations (vomiting, diarrhea, excessive sweating), the red blood cell count is within normal limits, but due to the loss of fluid affecting the blood plasma, the concentration of red blood cells is elevated.

Polycythemia due to stress

Stress-induced erythrocytosis is also known as pseudopolycythemia syndrome, which occurs in middle-aged obese men who take diuretics to treat hypertension. Often these same people are cigarette smokers.

Risk factors for polycythemia

The main risk factors for polycythemia are: chronic hypoxia; long-term cigarette smoking; family and genetic predisposition; life at high altitudes; prolonged exposure to carbon monoxide (tunnel workers, garage attendants, residents of heavily polluted cities); Ashkenazi Jews of Jewish origin (possibly increased incidence of polycythemia due to genetic predisposition).

Symptoms of polycythemia

The symptoms of polycythemia can vary widely. Some people with polycythemia have no symptoms at all. In polycythemia average, most of the symptoms are related to the underlying condition that is responsible for the polycythemia. The symptoms of polycythemia can be vague and quite general. Some of the important signs include: 1) weakness, 2) bleeding, blood clots (which can lead to heart attack, stroke, pulmonary embolism), 3) joint pain, 4) headache, 5) itching (also itchy after taking shower or bath), 6) fatigue, 7) dizziness, 8) abdominal pain.

When to see a doctor?

People with primary polycythemia should be aware of some of the potentially serious complications that can occur. Blood clots (heart attack, stroke, blood clots in the lungs or legs) and uncontrolled bleeding (nosebleeds, gastrointestinal bleeding) usually require immediate medical attention from your doctor.

Patients with primary polycythemia should be under the supervision of a hematologist. Diseases that lead to secondary polycythemia may be treated by a general practitioner or physicians from other specialties. For example, people with chronic lung disease should be seen regularly by a pulmonologist, and patients with chronic heart disease by a cardiologist.

Blood test for polycythemia

Diagnosing polycythemia is very easy with a routine blood test. When evaluating a patient with polycythemia, a complete medical examination is necessary. It is especially important to examine the lungs and heart. An enlarged spleen (splenomegaly) is a common feature in polycythemia. Therefore, it is very important to evaluate the enlargement of the spleen.

To assess the cause of polycythemia, it is important to conduct a complete blood count, determine the blood coagulation profile and metabolic panels. Other typical tests to determine possible causes of polycythemia include: chest x-ray, electrocardiogram, echocardiography, hemoglobin analysis, and carbon monoxide measurement.

In polycythemia vera, as a rule, other blood cells are also represented by an abnormally high number of white blood cells (leukocytosis) and platelets (thrombocytosis). In some cases, it is necessary to investigate the production of blood cells in the bone marrow, for this, an aspiration, or bone marrow biopsy, is performed. The guidelines also recommend testing for a JAK2 gene mutation as a diagnostic criterion for polycythemia vera. Erythropoietin level testing is not required, but in some cases this test can provide useful information. In the initial stage of polycythemia, the level of erythropoietin is usually low, but in an erythropoietin-secreting tumor, the level of this enzyme can be elevated. Results must be interpreted as erythropoietin levels may be high in response to chronic hypoxia (if this is the underlying cause of polycythemia).

Treatment of polycythemia

Treatment for secondary polycythemia depends on its cause. Supplemental oxygen may be needed for people with chronic hypoxia. Other treatments may be aimed at treating the cause of the polycythemia (eg, appropriate treatment for heart failure or chronic lung disease).

Individuals with primary polycythemia can take some home treatment measures to control symptoms and avoid possible complications. It is important to drink enough fluids to avoid further concentration of blood and dehydration. There are no restrictions on physical activity. If a person has an enlarged spleen, contact sports should be avoided to prevent injury to the spleen and rupture. It is best to avoid iron supplements, as their use can contribute to the overproduction of red blood cells.

Bloodletting (blood donation) remains the mainstay of therapy for polycythemia. The goal of bloodletting is to maintain a hematocrit of about 45% in men and 42% in women. Initially, it may be necessary to bleed every 2-3 days, removing 250 to 500 ml of blood each time. Once the goal is reached, bloodletting may not be performed as often.

The widely recommended drug for the treatment of polycythemia is hydroxyurea. This drug is especially recommended for people at risk of blood clots. Especially this drug is recommended for people over 70 years of age with an elevated platelet count (thrombocytosis; more than 1.5 million), with a high risk of cardiovascular disease. Hydroxyurea is also recommended for patients who are unable to tolerate bloodletting. Hydroxyurea can reduce all elevated blood counts (leukocytes, erythrocytes and platelets), while phlebotomy only reduces hematocrit.

Aspirin is also used in the treatment of polycythemia to reduce the risk of blood clots. However, this drug should not be used in people with any history of bleeding. Aspirin is usually used in combination with bloodletting.

Complications of polycythemia

Frequent monitoring is recommended at the start of treatment with phlebotomy to an acceptable hematocrit. Some of the complications of primary polycythemia, as listed below, often require constant medical supervision. These complications include: 1) a blood clot (thrombosis) causing a heart attack, blood clots in the legs or lungs, blood clots in the arteries. These events are considered the main causes of death in polycythemia; 2) severe blood loss or hemorrhage; 3) transformation into blood cancer (eg, leukemia, myelofibrosis).

Prevention of polycythemia

Many causes of secondary polycythemia cannot be prevented. However, there are some potential preventive measures: 1) smoking cessation; 2) avoid prolonged exposure to carbon monoxide; 3) timely treatment of chronic lung disease, heart disease or sleep apnea.

Primary polycythemia due to gene mutation is usually unavoidable.

Predictions for polycythemia. The prognosis for primary polycythemia without treatment is generally poor; with a life expectancy of about 2 years. However, with even a single bloodletting, many patients can lead normal lives and have a normal life expectancy. The outlook for secondary polycythemia depends largely on the underlying cause of the disease.

Hematologist

Higher education:

Hematologist

Samara State Medical University (SamSMU, KMI)

Level of education - Specialist
1993-1999

Additional education:

"Hematology"

Russian Medical Academy of Postgraduate Education

Polycythemia (erythremia, Wakez's disease, erythrocytosis) is a rare type of leukemia. It is characterized by an excessive concentration of red blood cells in the blood. Pathology can be primary and secondary - arising under the influence of certain factors. In fact, these are only symptoms, not independent diseases. But both forms of polycythemia are quite serious conditions, threatening serious consequences and life-threatening complications.

Polycythemia - what is it

With erythremia in the blood, the concentration of red blood cells and, accordingly, hemoglobin increases. Red blood cells are produced in the bone marrow. Erythropoietin, a hormone secreted to a greater extent in the kidneys and to a lesser extent in the liver, takes an active part in the process. The development of the primary (true) form of pathology is due to internal problems of erythrocyte secretion and is relatively rare.

Usually, the occurrence of erythremia provokes the progression of concomitant diseases - this is secondary polycythemia. The forms of the disease are different in severity of course and depending on the mechanisms of development of disorders in the hematopoietic organs. Polycythemia vera is the result of a benign tumor of the bone marrow, the development of which is due to the reproduction of immature red blood cells. The formation of a secondary form of pathology is influenced by circumstances that contribute to blood clotting:

dehydration of the body (diarrhea, periodic massive vomiting);
oxygen deficiency (being in the mountains, hot weather, high body temperature).

Oxygen and water starvation causes the body to make up for the deficiency due to increased secretion of red blood cells. They are able to perform their functions, their volume and shape are within the normal range. True polycythemia is characterized by a large number of cells that do not correspond to the norm in size and shape, that is, unnecessary to the body. Tumor growth is associated with two sets of cells:

developing from progenitor cells autonomously, as a result of gene mutation;
dependent on the influence of erythropoietin, which regulates the production of red blood cells.

The action of erythropoietin is activated when secondary erythremia "connects" to the development of the neoplasm. "Excess" red blood cells thicken the blood, increasing the risk of blood clots. Their excessive accumulation in the spleen, which is not capable of destroying such a number of aged erythrocytes, leads to an increase in the organ.

Reasons for the development of polycythemia

Excessive erythrocyte secretion is usually secondary. But there are regions (for example, Chuvashia) in which cases of familial erythremia are recorded, inherited in an autosomal recessive manner (true polycythemia appears only in homozygotes who have received one recessive gene from each parent). The main causes of the secondary form of erythremia are oxygen starvation of tissues (congenital and acquired) and a high content of erythropoietin in the blood. To increase the concentration of the hormone against the background of oxygen deficiency can:

pulmonary pathologies (emphysema, bronchitis);
increased pulmonary vascular resistance;
insufficient ventilation of the lung or its lobe;
heart failure;
sleep apnea syndrome;
poor blood supply to the kidneys;
frequent presence in the highlands.

Excessive amounts of erythropoietin secrete certain neoplasms:

liver;
adrenal glands;
kidneys;
uterus.

To increase the production of erythropoietin sometimes lead to obstruction of the kidneys and hepatic cysts, regular exposure to the tissues of organs of carbon monoxide (smoking). Polycythemia in newborns is often transmitted from the mother through blood transfusion or through the placenta. Prolonged intrauterine hypoxia can also provoke the development of erythremia in newborns.

Relative polycythemia is usually called a pathology in which the level of red blood cells in the blood increases due to dehydration. At the same time, the number of erythrocytes does not change - the volume of plasma (the liquid part of the blood) decreases, increasing the specific concentration of the remaining components of the blood. Heavy physical work and psycho-emotional stress can cause the so-called stress polycythemia, which is inherent in middle-aged men. In such a state, the number of cells also does not change, the volume of the liquid part of the blood decreases.

Signs of Wakez disease

True polycythemia, like the secondary one, proceeds extremely slowly, therefore the manifestations of the pathology are often weakly expressed or completely absent. Symptoms of polycythemia are usually non-specific:

distraction, weakness, tinnitus (lack of vital substances and oxygen in the cerebral vessels);
dizziness, heaviness in the head, headaches (stagnation of blood in the vessels of the brain associated with its increased viscosity);
vision problems (lack of oxygen and nutrients);
redness of the sclera of the eyes, mucous membranes, skin (accumulation of red blood cells in the superficial capillaries);
cyanosis of the skin (accumulation in the tissues of hemoglobin connected with carbon dioxide);
itching (high concentration of unbound bilirubin in the blood);
short-term severe pain in the fingertips (formation of small blood clots in the capillaries).

With the further development of polycythemic syndrome, note:

enlargement of the liver and spleen;
pain in the center of the sternum;
cardiopalmus;
pain in the bones;
high blood pressure;
thrombus formation;
acute violation of cerebral blood supply;
pronounced shortness of breath;
signs of a heart attack;
Mitchell's syndrome;
numbness and coldness of the extremities;
peptic ulcer;
flatulence;
negative changes in peripheral blood vessels.

Diagnosis of polycythemia

Diagnosis of pathology is carried out by a hematologist. At the same time, it is based on characteristic features combined into syndromes: plethoric ( due to an excess of blood in organs and tissues) and myeloproliferative (due to excessive activity of the bone marrow). The presence of polycythemia is indicated by an increased content in the blood:

erythrocytes (significant increase);
platelets (sometimes normal);
leukocytes (mainly neutrophils);
reticulocytes (immature red blood cells);
hemoglobin (a protein carried by red blood cells);
vitamin B 12.

There may be an increase in hematocrit - the ratio of the volume of red blood cells to the total volume of blood. True polycythemia is characterized by a low content of erythropoietin in the blood, with a secondary form of pathology, it is normal or increased.

Erythremia therapy

In the benign course of Wakez's disease, phlebotomy (bleeding) is sometimes prescribed. It is carried out every three days until the concentration of hemoglobin and red blood cells returns to normal. Bloodletting usually removes up to 400 ml of blood. Half an hour before the procedure, Heparin is administered intravenously to prevent the formation of blood clots and reduce blood viscosity.

Currently, bloodletting is losing ground, since there is a more effective method for removing excess red blood cells from the body - erythrocytapheresis. Using the hardware method, all red blood cells are removed from the blood (500-700 ml). Plasma and other blood components return to the bloodstream.

All methods of removing red blood cells from the blood are combined with the use of cytostatics (Imifos, Mielobramol) - drugs that inhibit the excessive formation of cells (including bone marrow tumor cells). The doctor prescribes a medication based on the clinical picture of the course of the pathology and analyzes of laboratory tests.

In addition to the use of cytostatic agents, physicians are increasingly resorting to the use of radioactive phosphorus. It accumulates in the bones, locally affecting the bone marrow. The drug significantly reduces the rate of blood formation (especially erythrocytes). As a rule, the drug is taken for a week 3-4 times a day. But the method has contraindications. It is impossible to use radioactive phosphorus, for example, with serious damage to the kidneys and liver.

Widely used to treat the pathology of hydroxyurea. This inhibitor is recommended for patients prone to thrombosis (over 70 years old, high platelet count, the presence of pathologies of the heart and blood vessels).

Polycythemia: home treatment

To adjust the content of red blood cells in the blood, you need to exclude eggs, milk and dairy products, red meat from the diet. Bad habits should be abandoned. Drinking even a small amount of alcohol increases the likelihood of thrombosis in the coronary vessels. Drugs negate medical therapy, and smoking increases blood pressure. In Wakez disease, this habit can cause sudden cardiac arrest.

To increase the effectiveness of the treatment of polycythemia, the menu will be very useful:

river fish (carp, pike perch, catfish);
legumes (peas, beans);
fruits (apricots, bananas, grapes);
vegetables (sauerkraut).

To thin the blood, a decoction of sweet clover is widely used. A teaspoon of herbs is poured with a glass of boiling water, filtered and drunk half a glass three times a day. Good blood thinner and cranberry tea. Fresh and dry berries are suitable for brewing. A glass of boiling water is poured over two tablespoons of berries, covered and insisted for about twenty minutes. Drink without restrictions, you can add honey. Not bad expand blood vessels and improve blood flow decoctions prepared on the basis of:

horse chestnut flowers;
periwinkle;
mushroom grass;
nettles;
burial ground.

Prevention of polycythemia

Most of the causes of polycythemia do not depend on the lifestyle of the patient. However, simple recommendations will help to postpone or reduce the manifestations of pathology, you should:

stop smoking;
drink enough fluids to prevent dehydration;
timely treat chronic pathologies of internal organs;
control body weight;
make time for physical exercise.

Physical activity is not limited, but with an enlarged spleen, the patient needs to give up contact sports - in order to avoid injury to the organ and its rupture. It is necessary to control the intake of iron supplements - they contribute to the increased production of red blood cells.

Polycythemia is characterized by benign development, but pathology is sometimes accompanied by serious complications. One of them is an embolism of the vessels of vital organs (liver, lungs, heart, brain), which can provoke a stroke or heart attack of internal organs. When symptoms of pathology appear, it is necessary to undergo a medical examination.

Polycythemia is a disease that can be assumed just by looking at the patient's face. And if you still conduct the necessary blood test, then there will be no doubt at all. In reference books, it can also be found under other names: erythremia and Wakez's disease.

Redness of the face is quite common and there is always an explanation for this. In addition, it is short-term and does not linger for a long time. Various reasons can cause sudden reddening of the face: fever, high blood pressure, a recent tan, an awkward situation, and emotionally labile people tend to blush often, even if others do not see any prerequisites for this.

Polycythemia is different. Here redness is persistent, not transient, evenly distributed over the entire face. The color of an overly “healthy” plethora is saturated, bright cherry.

What kind of disease is polycythemia?

True polycythemia (erythremia, Wakez's disease) belongs to the group of hemoblastoses (erythrocytosis) or chronic with a benign course. The disease is characterized by the growth of all three hematopoietic sprouts with a significant advantage of erythrocyte and megakaryocytic, due to which there is an increase not only in the number of red blood cells - but also in other blood cells that originate from these sprouts, where the source of the tumor process is the affected progenitor cells of myelopoiesis. It is they who begin uncontrolled proliferation and differentiation into mature forms of erythrocytes.

Most affected under such conditions are immature red blood cells, which are hypersensitive to erythropoietin even at low doses. With polycythemia at the same time, an increase in granulocytic leukocytes is also observed(primarily stab and neutrophils)and platelets. The cells of the lymphoid series, which include lymphocytes, are not affected by the pathological process, since they come from a different germ and have a different way of reproduction and maturation.

Cancer or not cancer?

Erythremia - not to say that it occurs all the time, however, there are a couple of people in a town of 25 thousand people, while for some reason men of 60 or so “love” this disease more, although any person can meet with such a pathology age. True, for newborns and young children, true polycythemia is absolutely not typical, therefore if erythremia is found in a child, then most likely she will wear secondary character and be a symptom and consequence of another disease (toxic dyspepsia, stress erythrocytosis).

For many people, a disease classified as leukemia (whether acute or chronic) is primarily associated with blood cancer. Here it is interesting to understand: is it cancer or not? In this case, it would be more expedient, clearer and more correct to talk about the malignancy or benignity of true polycythemia in order to determine the boundary between "good" and "evil". But, since the word "cancer" refers to tumors from epithelial tissues, then in this case this term is inappropriate, because this tumor comes from hematopoietic tissue.

Wakez disease refers to malignant tumors , but it is characterized by high differentiation of cells. The course of the disease is long and chronic, for the time being qualified as benign. However, such a course can only last up to a certain point, and then with proper and timely treatment, but after a certain period of time, when significant changes in erythropoiesis occur, the disease becomes acute and acquires more “evil” features and manifestations. Here it is - true polycythemia, the prognosis of which will completely depend on how quickly its progression occurs.

Why do sprouts grow incorrectly?

Any patient suffering from erythremia, sooner or later asks the question: "Why did this" disease happen "to me?". The search for the cause of many pathological conditions is usually useful and gives certain results, increases the effectiveness of treatment and promotes recovery. But not in the case of polycythemia.

The causes of the disease can only be assumed, but not definitely stated. Only one clue can be for the doctor when determining the origin of the disease - genetic abnormalities. However, the pathological gene has not yet been found, so the exact localization of the defect has not yet been determined. There are, however, suggestions that Wakez's disease may be associated with trisomy 8 and 9 pairs (47 chromosomes) or other violation of the chromosomal apparatus, for example, the loss of a section (deletion) of the long arm C5, C20, but this is still guesswork, although built on conclusions of scientific research.

Complaints and clinical picture

If there is nothing to say about the causes of polycythemia, then we can talk about clinical manifestations for a long time and a lot. They are bright and varied, since already from the 2nd degree of development of the disease, literally all organs are drawn into the process. Subjective sensations of the patient are of a general nature:

Weakness and constant feeling of fatigue;
Significant decrease in performance;
increased sweating;
Headaches and dizziness;
Marked memory loss;
Visual and auditory disorders (decrease).

Complaints characteristic of this disease and characterized by it:

Acute burning pain in the fingers and toes (the vessels are clogged with platelets and red blood cells, which form small aggregates there);
Soreness, though not so burning, in the upper and lower limbs;
Itching of the body (a consequence of thrombosis), the intensity of which increases markedly after a shower and a hot bath;
Periodic appearance of a rash such as urticaria.

It's obvious that cause all these complaints microcirculation disorder.

As the disease progresses, more and more new symptoms are formed:

skin and mucous membranes due to the expansion of capillaries;
Pain in the region of the heart, resembling;
Pain in the left hypochondrium caused by overload and enlargement of the spleen due to the accumulation and destruction of platelets and red blood cells (it is a kind of depot for these cells);
Enlargement of the liver and spleen;
Peptic ulcer of the stomach and 12 duodenal ulcer;
Dysuria (difficulty urinating) and pain in the lumbar region due to the development of uric acid diathesis, which was caused by a shift in the blood buffer systems;
Pain in bones and joints as a result hyperplasia(overgrowth) bone marrow;
Gout;
Manifestations of a hemorrhagic nature: bleeding (nose, gums, intestinal) and skin hemorrhages;
Injections of the vessels of the conjunctiva, which is why the eyes of such patients are called "rabbit eyes";
Tendency to and arteries;
shins;
Possible thrombosis of the coronary vessels with development;
Intermittent claudication, which may result in gangrene;
(almost 50% of patients), giving rise to a tendency to strokes and heart attacks;
Respiratory damage due to immune disorders, which cannot adequately respond to infectious agents that cause inflammation. In this case, red blood cells begin to behave like suppressors and suppress the immunological response to viruses and tumors. In addition, they are in the blood in an abnormally high amount, which further aggravates the state of the immune system;
The kidneys and urinary tract suffer, so patients have a tendency to pyelonephritis, urolithiasis;
The central nervous system does not remain aloof from the events taking place in the body, when it is involved in the pathological process, symptoms appear (with thrombosis), (less often), insomnia, memory impairment, mnestic disorders.

Asymptomatic to end stage

Since for polycythemia initially asymptomatic, the above manifestations do not occur in one day, but accumulate gradually and for a long time, it is customary to distinguish 3 stages in the development of the disease.

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

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

II A - proceeds without myeloid metaplasia of the spleen, subjective and objective symptoms of erythremia are present, the duration of the period is 10-15 years;

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

terminal stage, which has all the signs of a malignant process. Complaints of the patient are diverse, "everything hurts, everything is wrong." At this stage, cells lose their ability to differentiate, which creates a substrate for leukemia, which replaces chronic erythremia, or rather, it turns into acute leukemia.

The terminal stage is characterized by a particularly severe course (hemorrhagic syndrome, rupture of the spleen, infectious and inflammatory processes that cannot be treated due to deep immunodeficiency). It usually ends in death soon after.

Thus, the life expectancy for polycythemia is 15-20 years, which may be not bad, especially considering that the disease can overtake after 60. And this means that there is a certain prospect of living up to 80 years. However, the prognosis of the disease still depends most of all on its outcome, that is, on what form of leukemia erythremia is transformed into at stage III (chronic myeloid leukemia, myelofibrosis, acute leukemia).

Diagnosis of Wakez disease

The diagnosis of polycythemia vera is mainly based on laboratory data with the determination of the following indicators:

, in which you can notice a significant increase in red blood cells (6.0-12.0 x 10 12 / l), (180-220 G / l), (plasma and red blood ratio). The number of platelets can reach the level of 500-1000 x 10 9 /l, while they can significantly increase in size, and leukocytes - up to 9.0-15.0 x 10 9 /l (due to rods and neutrophils). with true polycythemia is always reduced and can reach zero.

Morphologically, erythrocytes do not always change and often remain normal, but in some cases, erythremia can be observed anisocytosis(erythrocytes of different sizes). Platelets indicate the severity and prognosis of the disease with polycythemia in the general blood test (the more there are, the more severe the course of the disease);

BAC (biochemical blood test) with level determination And . For erythremia, the accumulation of the latter is very characteristic, which indicates the development of gout (a consequence of Wakez's disease);
Radiological examination using radioactive chromium helps to determine the increase in circulating red blood cells;
Sternal puncture (bone marrow sampling from the sternum) followed by cytological diagnosis. In the preparation hyperplasia of all three sprouts with a significant predominance of red and megakaryocytic;
Trepanobiopsy(histological examination of material taken from the ilium) - the most informative method that allows you to most reliably identify the main symptom of the disease - three-growth hyperplasia.

In addition to hematological parameters, to establish the diagnosis of polycythemia vera, the patient is sent for an ultrasound examination (ultrasound) of the abdominal organs (enlargement of the liver and spleen).

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

And then the patient is waiting for treatment in the hematological department, where tactics are determined by clinical manifestations, hematological parameters and the stage of the disease. Therapeutic measures for erythremia usually include:

Bloodletting, which allows to reduce the number of red blood cells to 4.5-5.0 x 10 12 / l and Hb (hemoglobin) to 150 g / l. To do this, with an interval of 1-2 days, 500 ml of blood is taken until the number of erythrocytes and Hb drops. The bloodletting procedure is sometimes replaced by hematologists with erythrocytopheresis, when, after sampling by centrifugation or separation, red blood is separated, and the plasma is returned to the patient;
Cytostatic therapy (myelosan, imifos, hydroxyurea, hydroxycarbamide);
(aspirin, dipyridamole), which, however, require caution in use. So, acetylsalicylic acid can enhance the manifestation of hemorrhagic syndrome and cause internal bleeding if the patient has a stomach ulcer or duodenal ulcer;
Interferon-α2b, successfully used with cytostatics and increasing their effectiveness.

The treatment regimen for erythremia is signed by the doctor for each case individually, so our task is only to briefly acquaint the reader with the drugs used to treat Wakez's disease.

Nutrition, diet and folk remedies

A significant role in the treatment of polycythemia is assigned to the regime of work (reduction of physical activity), rest and nutrition. In the initial stage of the disease, when the symptoms are not yet expressed or are weakly manifested, the patient is assigned table number 15 (general), however, with some reservations. The patient is not recommended to consume products that enhance hematopoiesis(liver, for example) and offer to revise the diet, giving preference to dairy and vegetable products.

In the second stage of the disease, the patient is assigned table number 6, which corresponds to the gout diet and limits or completely excludes fish and meat dishes, legumes and sorrel. Having been discharged from the hospital, the patient must adhere to the recommendations given by the doctor during outpatient observation or treatment.

Question: "Is it possible to treat folk remedies?" sounds with the same frequency for all diseases. Erythremia is no exception. However, as already noted, the course of the disease and the life expectancy of the patient depend entirely on timely treatment, the purpose of which is to achieve a long and stable remission and delay the third stage for the longest possible time.

During the calm period of the pathological process, the patient must still remember that the disease can return at any time, therefore, he must discuss his life without exacerbation with the attending physician, in whom he is observed, periodically take tests and undergo an examination.

Treatment of blood diseases with folk remedies should not be generalized, and if there are many recipes for increasing hemoglobin levels or for, this does not mean at all that they are suitable for the treatment of polycythemia, from which, in general, medicinal herbs not yet found. Wakez's disease is a delicate matter, and in order to control the function of the bone marrow and thus affect the hematopoietic system, you need to have objective data that can be assessed by a person with certain knowledge, that is, the attending physician.

In conclusion, I would like to say to readers a few words about relative erythremia, which cannot be confused with the true one, since relative erythrocytosis can occur against the background of many somatic diseases and successfully end with the cure of the disease. In addition, erythrocytosis as a symptom can accompany prolonged vomiting, diarrhea, burn disease and hyperhidrosis. In such cases, erythrocytosis is a temporary phenomenon and is associated primarily with dehydration of the body, when the amount of circulating plasma, which consists of 90% water, decreases.