Polycythemia symptoms and treatment prognosis. What is polycythemia vera and is it treated? Etiology and clinic

Hepatosplenomegaly may also develop. Diagnosis is based on a complete blood count, testing for 1AK2 gene mutations, and clinical criteria. Treatment includes low-dose aspirin for all patients and myelosuppressive drugs for 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. The incidence in the United States is 1.9 / 100,000, with the risk increasing with age. PI is somewhat more common in men. PV is very rare in children.

Pathophysiology of polycythemia vera

With PI, there is an increased proliferation of all cell lines. In this regard, PIs are sometimes called panmyelosis due to the increase in the content of representatives of all 3 cell lines of peripheral blood. The increased production of one red blood cell lineage is called erythrocytosis. Isolated thrombocytosis can occur with PI, but more often it occurs for other reasons (secondary erythrocytosis).

Extraosseous 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 can go into a depletion 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 drugs and radioactive phosphorus. The latter should only be used rarely or not at all.

Complications. With PI, 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 has yet to be tested in prospective studies specially designed for this purpose.

Platelet function can be compromised, which increases the risk of bleeding. Accelerated cell turnover can increase the concentration of uric acid, thereby increasing the risk of gout and kidney stones.

Genetic factors. Clonal hematopoiesis is a hallmark of PI. 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 mutations) is found in virtually all PV patients. However, with almost complete certainty, it can be argued that there are other mutations underlying the disease. They keep the 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 accident by high hemoglobin or by symptoms of increased viscosity, such as fatigue, loss of concentration, headaches, dizziness, darkening of the eyes, itchy skin, nosebleeds. Sometimes it manifests itself with peripheral arterial disease or cerebrovascular disease. Patients are often plethoric, and in most patients an enlarged spleen is palpated. Thrombosis and often peptic ulcers, sometimes complicated by bleeding, may occur.

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, lightheadedness, visual impairment, fatigue, and shortness of breath. Itching is a common symptom, especially after showering. 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), may occur. Hepatomegaly is common, and splenomegaly (sometimes severe) occurs in 75% of patients.

Thrombosis can cause symptoms in the affected area (eg, neurologic pathology in stroke or transient ischemic attacks, leg pain, leg edema, or both in thrombosis of the lower extremities, unilateral vision loss in retinal thrombosis).

Bleeding occurs in 10% of patients.

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

Diagnosis of polycythemia vera

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

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

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

Examination of a bone marrow sample is not always necessary.

In those cases, when it is carried out, in the bone marrow, panmyelosis, a large size and crowding of megakaryocytes, usually attract attention. In some cases, reticulin fibers are found. However, no changes in the bone marrow make it possible to distinguish PI with absolute certainty from other pathological conditions (for example, congenital familial polycythemia) accompanied by erythrocytosis.

Plasma erythropoietin concentrations in PI patients are usually low or near the lower normal range. An increased concentration indicates a secondary nature of erythrocytosis.

In some cases, a study is carried out for the endogenous formation of erythroid cell colonies in vitro (erythrocyte precursors taken from peripheral blood or bone marrow of patients with PI, unlike those in healthy people, can form erythroid cells in culture without adding erythropoietin).

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

Non-specific abnormalities in laboratory parameters that may be observed with PI include an increase in the concentration of vitamin B 12 and an increase in B 12 - binding capacity, as well as hyperuricemia and hyperuricosuria (present in\u003e 30% of patients), increased expression of the PRV-1 gene in leukocytes, a decrease in the expression of the C-mpl gene (thrombopoietin receptor) in megakaryocytes and platelets. These tests are not required to establish a diagnosis.

Diagnosis of polycythemia is discussed in the Elevated Hemoglobin Subsection. For diagnosis, it is important to increase the mass of erythrocytes in the absence of reasons for secondary erythrocytosis and splenomegaly. Neutrophil and platelet counts are often increased, abnormal karyotype can be found in bone marrow, and in vitro culture of bone marrow shows autonomous growth in the absence of growth factors.

Prediction of polycythemia vera

In general, IP is associated with a shorter life span. The median survival rate for all patients is 8 to 15 years, although many live much longer. A common cause of death is thrombosis. The next most frequent are complications of myelofibrosis and the development of leukemia.

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

Treatment for polycythemia vera

• Aspirin treatment
• Possible bloodletting
• Possible myelosuppressive therapy.

Therapy should be selected individually, taking into account age, gender, health status, clinical manifestations and results of hematological studies. Patients are divided into high-risk and low-risk groups. High-risk patients include patients\u003e 60 years of age with a history of thrombosis or transient ischemic attacks, or both.

Aspirin. Aspirin reduces the risk of thrombosis. For this reason, patients undergoing only phlebotomy or phlebotomy should receive aspirin. Higher doses of aspirin carry an unacceptably high risk of bleeding.

Bloodletting. Bloodletting was the mainstay of treatment for patients in both high and low risk groups, 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 correlate with risk of thrombosis. Some clinicians no longer adhere to strict guidelines for phlebotomy. Bloodletting is still one of the possible alternatives for any patient. In a small proportion of patients with flushed skin and increased blood viscosity, bleeding may relieve symptoms. The standard threshold hematocrit above which phlebotomy is performed is\u003e 45% in men and\u003e 42% in women. As soon as the hematocrit value drops below the threshold, it is checked monthly and maintained at the same level by additional bloodletting, which is performed as needed. If necessary, the intravascular volume is replenished with crystalloid or colloidal solutions.

Myelosuppressive therapy is indicated for high-risk patients.

Radioactive phosphorus (32P) has long been used to treat PV. The effectiveness of treatment is 80 to 90%. Radioactive phosphorus is well tolerated and requires fewer clinic visits once disease control is achieved. However, the use of radioactive phosphorus is associated with an increased risk of developing acute leukemia. Leukemia following 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 concomitant pathology does not exceed 5 years). It should be prescribed only in rare cases. Many doctors don't use it at all.

Hydroxyurea inhibits the enzyme ribonucleoside diphosphate reductase. It is also used to suppress bone marrow activity. There are no unambiguous data on the ability of hydroxyurea to provoke leukemia. However, the likelihood of transformation into leukemia exists, although it is small. Patients have a weekly blood test. After reaching equilibrium, the intervals between blood tests are increased to 2 weeks, and then to 4 weeks. If the white blood cell count 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 the required level of blood cells cannot be maintained with the help of hydroxyurea, or when the latter is ineffective. It is worth noting that pegylated interferon alpha-2b is generally well tolerated. This drug acts on the disease at the molecular level and has relatively low toxicity.

Alkylating drugs can cause leukemia and should be avoided.

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

Treatment of complications. Hyperuricemia is corrected with allopurinol if an increase in uric acid concentration is accompanied by symptoms or if patients are simultaneously receiving myelosuppressive therapy. Itching can be tried to control with antihistamines, but this is sometimes difficult to achieve. Myelosuppression is often the most effective method. Examples of potentially effective therapy include cholestyramine, cyproheptadine, cimetedin, or paroxetine.

Bloodletting quickly relieves symptoms of hyperviscosity. 400-500 ml of blood is removed - and the venesection is repeated every 5-7 days until the hematocrit drops by 45%, removing 400-500 ml of blood with each procedure (less if the patient is elderly). Less frequent but regular phlebotomy maintains this level until hemoglobin decreases due to iron deficiency. The underlying myeloproliferation is suppressed by hydroxycarbamide or interferon. Treatment with radioactive phosphorus (5 mCi 32P 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 the size of the spleen, and reduce the transformation to myelofibrosis. Aspirin reduces the risk of thrombosis.

- chronic hemoblastosis, which is based on the unlimited proliferation of all germs of myelopoiesis, mainly erythrocytic. Clinically, polycythemia is manifested by cerebral symptoms (heaviness in the head, dizziness, tinnitus), thrombohemorrhagic syndrome (arterial and venous thrombosis, bleeding), microcirculatory disorders (chilliness of the limbs, erythromelalgia, flushing of the skin and mucous membranes). The main diagnostic information is obtained by examining peripheral blood and bone marrow. For the treatment of polycythemia, phlebotomy, erythrocytapheresis, and chemotherapy are used.

General information

Causes of polycythemia

The development of polycythemia is preceded by mutational changes in the pluripotent hematopoietic stem cell, which gives rise to all three bone marrow cell lines. The most common mutation of the JAK2 tyrosine kinase gene with the replacement of valine with phenylalanine at position 617. Occasionally there is a familial incidence of erythremia, for example among Jews, which may support a genetic correlation.

In polycythemia, there are 2 types of erythroid hematopoietic precursor cells in the bone marrow: some of them behave autonomously, their proliferation is not regulated by erythropoietin; others, as expected, are erythropoietin-dependent. It is believed that the autonomous population of cells is nothing more than a mutant clone - the main substrate of polycythemia.

In the pathogenesis of erythremia, the leading role belongs to enhanced erythropoiesis, which results in absolute erythrocytosis, impaired rheological and coagulating properties of blood, myeloid metaplasia of the spleen and liver. High blood viscosity leads to a tendency to vascular thrombosis and hypoxic tissue damage, and hypervolemia causes increased blood circulation in the internal organs. At the end of polycythemia, there is a depletion of hematopoiesis and myelofibrosis.

Classification of polycythemia

In hematology, there are 2 forms of polycythemia - true and relative. Relative polycythemia develops with a normal red blood cell count and a decrease in plasma volume. This condition is called stressful or pseudo-polycythemia and is not covered in this article.

Polycythemia vera (erythremia) can be primary or secondary in origin. The primary form is an independent myeloproliferative disease, which is based on the defeat of the myeloid line of hematopoiesis. Secondary polycythemia usually develops with increased activity of erythropoietin; this condition is a compensatory reaction to general hypoxia and can occur in chronic pulmonary pathology, "blue" heart defects, adrenal tumors, hemoglobinopathies, when climbing to a height or smoking, etc.

Polycythemia vera in its development goes through 3 stages: initial, extended and terminal.

Stage I (initial, oligosymptomatic) - lasts about 5 years; is asymptomatic or with minimal clinical manifestations. It is characterized by moderate hypervolemia, slight erythrocytosis; the size of the spleen is normal.

Stage II (erythremic, extended) is divided into two sub-stages:

• IA - without myeloid transformation of the spleen. Erythrocytosis, thrombocytosis, and sometimes pancytosis are noted; according to myelogram data - hyperplasia of all hematopoietic germs, pronounced megakaryocytosis. The duration of the extended stage of erythremia is 10-20 years.
• IIB - with the presence of myeloid metaplasia of the spleen. Hypervolemia, hepato- and splenomegaly are expressed; in peripheral blood - pancytosis.

III stage (anemic, post-erythremic, terminal). Characterized by anemia, thrombocytopenia, leukopenia, myeloid transformation of the liver and spleen, secondary myelofibrosis. Possible outcomes of polycythemia in other hematological malignancies.

Symptoms of polycythemia

Erythremia develops for a long time, gradually and can be detected by chance when examining blood. Early symptoms, such as heaviness in the head, tinnitus, dizziness, blurred vision, chilliness of the extremities, sleep disturbance, etc., are often attributed to old age or concomitant diseases.

The most characteristic feature of polycythemia is the development of plethoric syndrome caused by pancytosis and an increase in BCC. Telangiectasia, cherry-red color of the skin (especially the face, neck, hands and other open areas) and mucous membranes (lips, tongue), hyperemia of the sclera are evidence of plethora. A typical diagnostic sign is Kuperman's symptom - the color of the hard palate remains normal, and the soft palate acquires a stagnant cyanotic hue.

Another hallmark symptom of polycythemia is itchy skin, which is worse after water treatments and is sometimes unbearable. Specific manifestations of polycythemia also include erythromelalgia - a painful burning sensation in the tips of the fingers, which is accompanied by their hyperemia.

In the expanded stage of erythremia, painful migraines, bone pain, cardialgia, and arterial hypertension may occur. In 80% of patients, moderate or severe splenomegaly is found; the liver enlarges somewhat less frequently. Many patients with polycythemia notice increased bleeding of the gums, bruising on the skin, prolonged bleeding after tooth extraction.

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

The result of microthrombosis and a violation of the trophism of the skin and mucous membranes are trophic ulcers of the leg, ulcers of the stomach and duodenum. The most frequent complications in the clinic of polycythemia are in the form of vascular thrombosis of deep veins, mesenteric vessels, portal veins, cerebral and coronary arteries. Thrombotic complications (PE, ischemic stroke, myocardial infarction) are the leading causes of death in patients with polycythemia. At the same time, along with thrombus formation, patients with polycythemia are prone to hemorrhagic syndrome with the development of spontaneous bleeding of various localizations (gingival, nasal, from the veins of the esophagus, gastrointestinal, etc.).

Diagnosis of polycythemia

Hematological changes that characterize polycythemia are decisive in the diagnosis. Blood tests reveal erythrocytosis (up to 6.5-7.5x10 12 / l), increased hemoglobin (up to 180-240 g / l), leukocytosis (over 12x10 9 / l), thrombocytosis (over 400x10 9 / l). The morphology of erythrocytes, as a rule, is not changed; with increased bleeding, microcytosis may be detected. An increase in the mass of circulating erythrocytes more than 32-36 ml / kg of a neurologist, cardiologist, gastroenterologist, urologist serves as a reliable confirmation of erythremia.

Treatment and prognosis of polycythemia

In order to normalize the volume of the BCC and reduce the risk of thrombotic complications, bloodletting is the first measure. Exfusion of blood is carried out in a volume of 200-500 ml 2-3 times a week, followed by replenishment of the removed blood volume with saline or rheopolyglucin. Frequent bloodletting may result in the development of iron deficiency anemia. Bloodletting in polycythemia can be successfully replaced by erythrocytapheresis, which allows only the erythrocyte mass to be removed from the bloodstream, returning the plasma.

In the case of pronounced clinical and hematological changes, the development of vascular and visceral complications, they resort to myelodepressive therapy with cytostatics (busulfan, mitobronitol, cyclophosphamide, etc.). Radioactive phosphorus therapy is sometimes given. To normalize the aggregate state of the blood, heparin, acetylsalicylic acid, dipyridamole are prescribed under the control of a coagulogram; with hemorrhages, platelet transfusions are indicated; with urate diathesis - allopurinol.

The course of erythremia is progressive; the disease is not prone to spontaneous remissions and spontaneous healing. Patients are forced to be under the supervision of a hematologist for life, to take courses of hemoexfusion therapy. With polycythemia, the risk of thromboembolic and hemorrhagic complications is high. The frequency of transformation of polycythemia to leukemia is 1% in patients who did not receive chemotherapy treatment, and 11-15% in those who received cytostatic therapy.

Erythremia (polycythemia vera, Vakez's disease) is a hereditary disease of the blood system that occurs mainly in elderly women.

Increased red blood cell count in polycythemia vera

This pathology is characterized by malignant hypertrophy of the bone marrow. Most often, this pathology is known to patients as blood cancer (although such a judgment is erroneous) and leads to a progressive increase in the number of blood cells, primarily erythrocytes (the number of other elements also increases). As a result of an increase in their number, an increase in hematocrit is observed, which leads to a decrease in the rheological properties of blood, a decrease in the speed of blood flow through the vessels, and as a consequence, an increase in thrombus formation and a deterioration in tissue supply.

These reasons lead to the fact that most tissues experience oxygen starvation, which reduces their functional activity (ischemic syndrome). Polycythemia vera occurs primarily in women. Men get sick a little less often, the incidence of this pathology is approximately 3: 2.

On average, Vakez disease occurs around the age of 40, and symptoms peak between 60 and 70 years of age. There is a hereditary predisposition to the disease. In the population, erythremia is quite rare - about 30 cases per million of the population.

The main symptoms of the disease

Erythremia is an excessive saturation of the blood with red blood cells, which leads to various tissue and vascular disorders. Among the most common symptoms are:

1. Skin discoloration. The main reasons are blood stasis and hemoglobin recovery. Due to the reduced blood flow, erythrocytes stay in one place longer, which leads to the restoration of the hemoglobin contained in them, and as a consequence, a change in the color of the skin. Patients suffering from this disease have a characteristic appearance - an intensely cherry-colored reddened face and neck. In addition, swollen veins are clearly visible under the skin. When studying mucous membranes, one can observe a characteristic symptom of Kuperman - a change in the color of the soft palate with an unchanged color of the hard one.
2. Itching. This syndrome develops as a result of an increase in the number of immune cells that are capable of releasing specific inflammatory mediators, in particular, serotonin and histamine. Itching worsens after mechanical contact (most often after a shower or bath).
3. Erythromelalgia - discoloration of the distal phalanges of the fingers with the appearance of pain... This syndrome is caused by an increased content of platelets in the blood, which leads to clogging of the small capillaries of the distal phalanges, the development of an ischemic process and pain in their tissues.
4. Spleno- and hepatomegaly.An increase in these organs is observed in most hematological diseases. If a patient develops erythremia, then an increased concentration of cells in the blood can lead to an increase in blood flow in these organs, and as a result, their increase. This can be determined by palpation or instrumental studies. Megalovirus syndrome is independently eliminated after the normalization of hemogram indicators, that is, when the blood test returns to normal.
5. Thrombosis. Due to the high concentration of cells in the blood and a decrease in blood flow, a large number of blood clots form at the sites of vascular intimal lesions, which lead to blockage of blood vessels in all parts of the body. The development of thrombosis of mesenteric, pulmonary or cerebral vessels is especially dangerous. In addition, blood clots in small vessels of the gastric mucosa lead to a decrease in its protective properties and the appearance of gastritis and ulcers. DIC syndrome may also occur.
6. Pain. It can develop as a result of vascular disorders, for example, with obliterating endarteritis, and as a result of some metabolic disorders. With polycythemia, there may be an increase in the level of uric acid in the blood, its deposition in the joints. In rare cases, pain syndrome is observed during percussion or beatings of flat bones containing bone marrow (due to its hyperplasia and stretching of the periosteum).

Among the general symptoms, if erythremia occurs, headache, dizziness, a feeling of heaviness in the head, tinnitus, a syndrome of general weakness come first (all symptoms are due to a decrease in tissue oxygenation, impaired blood circulation in certain parts of the body). When diagnosing, they are not used as mandatory criteria, since they can correspond to any systemic disease.

Stages and degrees of polycythemia

Polycythemia vera has three stages (stages):

• stage of initial manifestations... At this stage, the patient makes no specific complaints. He is worried about general weakness, increased fatigue, and a feeling of discomfort in the head. All these symptoms are most often attributed to overwork, social and life problems, due to which the disease itself is diagnosed rather late;
• extended stage (clinical stage)... At this stage, the appearance of a headache, discoloration of the skin and mucous membranes is characteristic. The pain syndrome develops quite late and speaks of the neglect of the disease;
• terminal stage... At this stage, the lesions of internal organs due to their ischemia, dysfunction of all body systems are manifested as much as possible. Death may occur due to secondary pathology.

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

Diagnosis of Vakez disease

For the diagnosis, a complete blood count plays a decisive role. It exhibits pronounced erythrocytosis, an increase in hemoglobin and hematocrit. The most reliable is the analysis of bone marrow punctate, in which signs of hyperplasia of the erythroid lineage are detected, and it is also calculated how many cells are present in it and what is their morphological distribution.

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

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

Erythremia treatment

Despite the variety and severity of Wakez disease, there are relatively few treatments available. It depends on what the analysis of the hemogram has shown, whether the cytological syndrome has developed and what symptoms the patient has.

As mentioned above, the cause of the disease is an increased concentration of blood cells (especially erythrocytes), which develops due to bone marrow hyperplasia. In this regard, the correct analysis of the pathways of the development of the disease makes it possible to determine the basic principles of pathogenetic treatment, which include a decrease in the number of blood cells and the effect directly on the places of their formation. This is achieved through the following treatments:

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

It is also recommended to add some cytostatic drugs to the treatment regimen (if cancer is the cause of bone marrow hyperplasia), interferons (in the development of secondary viral complications) or hormones (mainly dexamethasone and prednisolone are used), which improves the prognosis of the disease.

Complications, consequences and prognosis

All complications of the disease are due to the development of vascular thrombosis. As a result of their blockage, infarctions of internal organs (heart, liver, spleen, brain), obliterating atherosclerosis (with thrombosis of the vessels of the lower extremities affected by atherosclerotic plaques) can develop. An excess of hemoglobin in the blood provokes the development of hemochromatosis, urolithiasis or gout.

All of them develop secondarily and require elimination of the main cause - erythrocytosis, for the most effective cure.

As for the prognosis of the disease, much here depends on the age at which treatment was started, what methods were used, and whether they were effective.

As mentioned at the beginning, polycythemia vera tends to develop later. If the appearance of the main symptoms is observed in young people (aged 25 to 40), then the disease is malignant, that is, the prognosis is poor, and secondary complications develop much faster. Accordingly, the later the development of the disease is observed, the more benign it is. When adequately prescribed drugs are used, the duration and life of patients is significantly improved. Such patients can live normally with their disease for quite a long time (up to several decades).

Answering the question of what the outcome of erythremia can be, it should be noted that everything depends on:

• what secondary processes have developed
• what are their reasons
• how long have they been
• whether polycythemia vera was diagnosed in a timely manner and the necessary treatment started.

Most often, due to damage to the liver and spleen, there is a transition of polycythemia to a chronic form of myeloid leukemia. The life span with it remains almost the same, and with the correct selection of drugs it can reach tens of years (the prognosis is relatively

Among blood diseases, there are many that cause a decrease in various elements - erythrocytes, leukocytes, platelets. But in some pathologies, on the contrary, there is an uncontrolled increase in the number of blood cells. The condition in which there is a chronic increase in the number of red blood cells, and other pathological changes occur, is called "polycythemia vera".

Features of the disease

Primary (true) polycythemia is a blood disease from the leukemia group that occurs idiopathically (for no apparent reason), lasts a long time (chronic) and is characterized by an increase in the number of red blood cells, an increase in hematocrit and blood viscosity. Synonyms for the name of the pathology are Vakez-Osler disease, erythremia, primary erythrocytosis. The consequences of erythrocytosis and blood clotting in this myeloproliferative disease can be serious and relate to the risk of thrombosis, an increase in the size and malfunction of the spleen, an increase in the volume of circulating blood, etc.

Erythremia is considered a malignant neoplastic process, which is caused by increased proliferation (hyperplasia) of bone marrow cells. The pathological process is especially strong for the erythroblastic sprout - a part of the bone marrow, consisting of erythroblasts and normoblasts. The pathogenesis of the main manifestations is associated with the appearance in the blood of a huge number of erythrocytes, as well as with a slight increase in the number of platelets and neutrophils (neutrophilic leukocytes). At the same time, blood cells are morphologically normal, but their number is abnormal. As a result, blood viscosity and the amount of blood in the circulating bloodstream increase. The result is a slower blood flow, the formation of blood clots, a violation of the local blood supply to tissues and their hypoxia.

If initially the patient most often has primary erythrocytosis, that is, only the number of erythrocytes increases, then further changes begin to cover other blood cells. Extramedullary hematopoiesis (pathological formation of blood outside the bone marrow) occurs in the organs of the peritoneum - in the liver and spleen, where part of erythropoiesis - the process of formation of erythrocytes - is also localized. At a late stage of the disease, the life cycle of erythrocytes is shortened, anemia, thrombocytopenia, myelofibrosis can develop, and the precursor cells of leukocytes and erythrocytes enter the general bloodstream without maturing. In about 10% of cases, the pathology flows into acute leukemia.

The study and the first description of erythrocytosis was made in 1892 by Vakez, and in 1903 the scientist Osler suggested that the cause of the disease is a violation of the bone marrow. Polycythemia vera is observed somewhat more often than other similar pathologies, but it is still quite rare. It is diagnosed in about 5 people per year per 1 million of the population. Most often, the disease is observed in people over 50 years old, the average age of detection is 60 years. In children, this diagnosis is very rare, mainly after 12 years. On average, only 5% of cases are under 40 years of age. Men suffer from this pathology more often than women. In the general structure of chronic myeloproliferative diseases, polycythemia vera ranks 4th. Sometimes it is inherited, so there are family cases.

Causes of pathology

The primary form of the disease is considered hereditary, transmitted in an autosomal recessive manner. In this case, it is often referred to as "family polycythemia." But most often, erythremia is a secondary condition, representing one of the manifestations of a general pathological process. The exact causes have not been established, but there are several theories about the appearance of polycythemia vera. Thus, there is a connection between the development of the disease and the transformation of stem cells, when a tyrosine kinase mutation occurs, which occurs in polycythemia vera more often than in other blood diseases.

Studies of cells with erythremia revealed in many patients the clonal origin of the pathology, since the same enzyme was detected in leukocytes, platelets, erythrocytes. The clonal theory is also confirmed by the ongoing cytological studies in relation to the karyotype of chromosome groups, where various defects that are similar in different patients were identified. There is also a viral genetic theory, according to which up to 15 types of viruses can enter the body and, with the participation of a number of provoking factors, lead to a malfunction of the bone marrow. They penetrate into the precursors of blood cells, which then, instead of normal maturation, begin to divide and form new red blood cells and other cells.

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

• lung disease;
• long stay at a high altitude above sea level;
• pulmonary hypoventilation syndromes;
• different hemoglobinopathies;
• long experience of smoking;
• tumors of the bone marrow, blood;
• hemoconcentration with long-term use of diuretics;
• burns to a large part of the body;
• severe stress;
• diarrhea;
• exposure to x-rays, radiation;
• poisoning with vapors of chemicals, their penetration through the skin;
• intake of toxic substances in the digestive tract;
• treatment with gold salts;
• advanced tuberculosis;
• serious surgical interventions;
• "Blue" heart defects;
• kidney pathology - hydronephrosis, renal artery stenosis.

Thus, the main cause of secondary erythrocytosis is all conditions that one way or another provoke tissue hypoxia, stress for the body or its intoxication. In addition, oncological processes, endocrine pathologies, and liver diseases can have a great effect on the brain and the production of additional blood cells.

Classification of polycythemia vera

The disease is classified into the following stages:

1. First, or initial stage. It can last for more than 5 years, represents the development of plethoric syndrome, that is, increased blood circulation in organs. At this stage, symptoms may be mild, no complications arise. A general blood test reflects a slight increase in the number of erythrocytes, a bone marrow puncture shows an increase in erythropoiesis or the production of all basic blood elements, with the exception of lymphocytes.
2. The second is the A stage, or polycythemic stage. Duration - from 5 to 15 years. Pletoric syndrome is more pronounced, there is an increase in the spleen, liver (hematopoietic organs), thrombus formation in the veins and arteries is often recorded. Tumor growth in the peritoneal organs is not observed. If this stage ends with a decrease in the number of platelets - thrombocytopenia, then the patient may experience various bleeding. Frequent hemorrhages cause a lack of iron in the body. A complete blood count reflects an increase in erythrocytes, platelets, leukocytes, with an advanced course - a decrease in platelets. In the myelogram, there is an increased formation of most blood cells (with the exception of lymphocytes), cicatricial changes in the brain are formed.
3. The second is stage B, or stage polycythemic with myeloid metaplasia of the organ - the spleen. The patient continues to increase the size of the spleen, and often the liver. Puncture of the spleen reveals tumor growth. There are frequent thrombosis, alternating with bleeding. In the general analysis, there is an even greater increase in the number of erythrocytes, leukocytes, there are erythrocytes of different sizes, shapes, and immature precursors of all blood cells are present. In the bone marrow, the number of cicatricial changes increases.
4. Third, or anemic stage. It is an outcome of a disease in which the activity of blood cells is depleted. The number of erythrocytes, leukocytes, and platelets is greatly reduced, the liver and spleen are enlarged with myeloid metaplasia, and extensive scarring occurs in the bone marrow. A person gets disability, most often - due to the consequences of thrombosis or the addition of acute leukemia, myelofibrosis, hypoplasia of hematopoiesis or chronic myeloid leukemia. This stage is recorded approximately 10-20 years after the development of the pathology.

Symptoms of manifestation

Often, this pathology is asymptomatic, but only at its initial stages. Later, the disease in the patient manifests itself in one way or another, while the specific symptoms can be varied. Basically, the symptom complex includes the following main signs:

1. Change in skin tone, varicose veins. Most often, in the neck area of \u200b\u200ban adult, veins begin to show through strongly, their pattern becomes stronger due to swelling, overfilling with blood. But the most obvious are the skin signs: the color of the skin becomes dark red, literally cherry. This is most noticeable in the area of \u200b\u200bthe neck, arms, face, which is associated with overfilling of the subcutaneous arteries with blood. At the same time, many patients mistakenly think that blood pressure rises against the background of hypertension, and therefore they often continue to take drugs for pressure and do not go to the doctor. With a careful attitude to health, you can notice that the lips and tongue also changed their shade, became red-blue. The blood vessels of the eyes are also poured, their plethora leads to hyperemia of the sclera and conjunctiva of the organs of vision. The hard sky remains the same color, but the soft sky also becomes brighter, burgundy.
2. Itchy skin. All the described changes on the part of the skin in about half of the cases are complemented by severe discomfort and itching. This symptom is very characteristic of erythremia, both primary and secondary. Since after taking water procedures in patients, histamine, as well as prostaglandins, are released, itching of the skin may become even more pronounced after a bath or shower.
3. Pain in the limbs. Many people develop obliterating endarteritis, resulting in persistent and severe pain in the legs. They can increase with exertion, prolonged walking, in the evening, at first they are often perceived as a symptom of fatigue in an elderly person. Pain is also observed on palpation and tapping of flat bones, which reflects the process of hyperplasia and cicatricial changes in the bone marrow. The next type of pain in a person with polycythemia vera is persistent burning pains in the area of \u200b\u200blarge and small joints of the legs, which resemble gouty pains and are caused by the same reason as gout - an increase in the level of uric acid. Another type of pain is severe, poorly tolerated pain in the fingers and toes, in which the skin becomes bluish-red, blue spots appear on it. These pains are caused by an increase in the number of platelets and the appearance of microthrombosis of the capillaries.
4. Splenomegaly. The growth of the spleen in size is observed in almost every person with polycythemia vera, but at different stages of the disease. This is due to the increased filling of the spleen with blood and the development of myeloproliferative phenomena. Somewhat less often, but still there is a strong increase in the size of the liver - hepatomegaly.
5. Peptic ulcer disease. About one in ten people with Wakez-Osler disease develop ulcers in the small intestine (usually in the duodenum) and in the stomach. This is due to the activation of Helicobacter pylori bacteria, as well as the development of microthrombosis in the gastrointestinal tract.
6. Thrombosis and bleeding. Almost all patients have a tendency to thrombosis at a certain stage, and even until recently, patients died from such complications at an early stage of the disease. The current modern treatment can prevent the appearance of blood clots in the brain, spleen, and legs, which threaten with embolism and death. Increased blood viscosity characterizes polycythemia vera in the initial stages, and later, against the background of depletion of the platelet formation system, bleeding develops - it is observed in the gums, nose, uterus, and gastrointestinal tract.

There are other signs of polycythemia vera that a person may complain about, but they are not very specific and may be inherent in different pathologies:

• fatigue;
• head legs;
• tinnitus;
• nausea;
• dizziness;
• a feeling of pulsation in the temples, ears;
• decreased appetite, performance;
• the appearance of "flies" before the eyes;
• other visual impairments - loss of fields, drop in visual acuity;
• shortness of breath, coughing;
• increased blood pressure;
• unexplained weight loss;
• prolonged subfebrile condition;
• insomnia;
• numbness, pinching of the fingers;
• epileptiform seizures and paralysis (rare).

In general, the disease is characterized by a long and sometimes benign course, especially with adequate treatment. But some people, especially those not receiving therapy, may have early onset of various consequences of polycythemia vera.

Possible complications

Most often, complications are associated with thrombosis and embolism of veins and blood vessels of the spleen, liver, legs, brain, and other areas of the body. This leads to different consequences depending on the size of the thrombus, the affected area. Transient ischemic attacks, strokes, thrombophlebitis and phlebothrombosis of superficial and deep veins, blockage of retinal vessels by thrombus and blindness, infarctions of internal organs, myocardial infarction may occur.

In the most advanced stages of the pathology, kidney stones (urolithiasis), gout, nephrosclerosis, and cirrhosis of the liver often appear. Complications are likely due to tissue bleeding - bleeding from gastrointestinal ulcers, anemia. On the part of the heart, in addition to myocardial infarction, signs of myocardiosclerosis and heart failure may also appear. There is also the likelihood of the transition of polycythemia vera to acute leukemia, chronic leukemia and other oncopathologies.

Diagnostics

It is difficult to diagnose this disease, especially in the absence of a characteristic clinical picture and in the presence of only general symptoms. Nevertheless, the totality of data from hematological and biochemical analyzes, as well as some distinctive features of the patient's appearance, coupled with his complaints, will help the doctor determine the cause of the changes taking place.

The main indicators for the diagnosis of polycythemia vera are indicators of a general blood test - the number of erythrocytes and hematocrit. In men, the development of this disease can be suspected if the number of erythrocytes is more than 5.7 * 10 * 9 / l, hemoglobin is more than 177 g / l, and the hematocrit is above 52%. In women, an excess of indicators is noted if they are more than 5.2 * 10 * 9 / l, 172 g / l, 48-50%, respectively. These figures are typical for the early stages of pathology, and as it develops, they become even higher. In addition, it is important to assess the mass of circulating erythrocytes, which normally for men will be up to 36 ml / kg, for women - up to 32 ml / kg.

Other blood parameters (by biochemistry, general analysis and other analyzes), which, in combination with the described disorders and in combination with each other, reflect the picture of the development of primary or secondary erythrocytosis:

1. Moderate or severe thrombocytosis (above 400 * 10 * 9 l), neutrophilic leukocytosis (above 12 * 10 * 9 l) with the presence of an increased number of basophils and eosinophils.
2. An increase in the number of reticulocytes.
3. The appearance in the blood of myelocytes, metamyelocytes.
4. Increase in blood viscosity by 500-800%.
5. Strong decrease in ESR.
6. An increase in the mass of circulating erythrocytes.
7. Increase in alkaline phosphatase, vitamin B12 in serum.
8. An increase in the amount of uric acid in the serum.
9. The oxygen saturation in the arteries is above 92%.
10. The appearance of erythrocyte colonies in a test tube.
11. Decreased erythropoietin index.
12. Change in color score less than 1.

At the stage of myelofibrosis, the indicators of hemoglobin and erythrocytes may return to normal, but the number of leukocytes increases greatly, their immature forms appear, and the presence of erythroblasts is diagnosed. As for the myelogram, which is obtained by puncturing the bone marrow, the following changes are revealed in it:

• reducing the presence of fatty inclusions;
• increased erythroblasts, normoblasts;
• hyperplasia of myelopoiesis germs.

There are other criteria by which the doctor can conclude about the ongoing changes characteristic of polycythemia vera:

1. Hepatosplenomegaly.
2. Tendency to thrombosis.
3. Excessive sweating combined with weight loss, weakness.
4. The presence of gene abnormalities if genetic testing has been performed when it comes to primary erythremia.
5. Increase in the average amount of circulating blood.

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

Treatment methods

The earlier a person seeks help, the more effective the therapy can be. At the third stage, or with the layering of another tumor process on erythremia, symptomatic therapy is carried out in combination with treatment with chemotherapy drugs. Chemotherapy treatment can be recommended at other stages of the disease, but the body does not always give an adequate response to it. Of the symptomatic remedies that improve the quality of life, the following are used:

1. Medicines against high blood pressure, mainly from the group of ACE inhibitors.
2. Antihistamines for itching, skin irritation, and other allergic reactions.
3. Antiplatelet and anticoagulants for blood thinning with a tendency to thrombus formation.
4. Local and systemic haemostatic agents for tissue bleeding.
5. Medicines to lower the uric acid level.

Treatment options for polycythemia vera include:

1. Bloodletting, or removing a small amount of blood from the bloodstream (phlebotomy). As a rule, they are made in a volume of 100-400 ml (according to indications) and a break of 3-4 days in a course of several sessions. The blood after such manipulations becomes more liquid, but they cannot be done in the presence of blood clots in a recent history. Before treatment with bloodletting, the patient is injected with a solution of Reopolyglucin, as well as Heparin.
2. Erythrocytaparesis. It is used to cleanse the blood from excess red blood cells, as well as platelets. Such sessions are done once a week.
3. Chemotherapy. It is used, as a rule, when the disease reaches the tumor stage - the second B. Other indications for chemotherapy are the presence of complications from the peritoneal organs, the general difficult situation of a person, an increase in the number of all blood elements. For chemotherapy, or cytoreductive therapy, cytostatics, antimetabolites, alkylating drugs, and biological drugs are used. The most commonly prescribed drugs are Leukeran, Hydroxyurea, Mielosan, and recombinant interferon.
4. Treatment of iron deficiency with androgens, erythropoietin, which are most often used in combination with glucocorticosteroids.
5. Radiation therapy. It is used to irradiate the spleen area and stop the cancer process in it, it is used with a strong increase in the size of the organ.
6. Transfusion of erythrocyte mass from purified erythrocytes. Used for severe anemia up to coma. If thrombocytopenia develops in the final stages of polycythemia vera, donor platelet transfusion may be necessary.

Bone marrow transplantation for a disease such as erythremia often leads to unfavorable results, therefore it is rarely used. In some cases, splenectomy is indicated, but with the development of acute leukemia, such an operation is not performed even with severe splenomegaly.

Features of treatment in pregnant women

This pathology rarely occurs during pregnancy. However, in the presence of a predisposition (hereditary or from secondary factors), pregnancy, childbirth and abortion can become a trigger for the development of polycythemia vera. Pregnancy always worsens the course of this disease, and its outcome can be more severe than outside of gestation. Nevertheless, in 50% of cases, pregnancy ends in a successful birth. The remaining half is due to miscarriages, developmental delay, fetal body anomalies.

Treatment of the disease in pregnant women is not easy. Most drugs are strictly contraindicated, as they have pronounced teratogenic properties. Therefore, during pregnancy, mainly bloodletting therapy and, if necessary, glucocorticosteroids are performed. For the prevention of complications and early detection of the disease in pregnant women, blood tests should be carried out regularly according to the schedule indicated by the observing obstetrician-gynecologist.

What not to do

It is categorically impossible to use diuretics, which additionally thicken the blood. Also, in our time, the use of radioactive phosphorus preparations is limited, which seriously inhibit myelopoiesis and often lead to the development of leukemia. Also, you cannot maintain the same nutritional system: the diet must change. All foods that enhance blood formation are prohibited, such as the liver. It is better to form a diet like a dairy-plant, and refuse excess meat.

The patient should not overload the body, engage in heavy sports, ignore regular rest. Treatment with folk remedies can be used, but only after a thorough examination of all the remedies by a physician in terms of composition, in order to prevent an increase in the production of red blood cells. Most often, symptomatic therapy is used to remove uric acid, reduce pain and itching of the skin, etc.

Prevention and prognosis

Prevention methods have not yet been developed. The prognosis for life differs depending on the severity of the course of the disease. Without treatment, up to a third of patients die within the first 5 years from the date of diagnosis. If a full-fledged therapy is carried out, then a person's life can be extended to 10-15 years or more. The most common cause of death is thrombosis, and only occasionally do people die from blood cancer (leukemia) or heavy bleeding.

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 normal limits.

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 a slowdown in 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 of the skin, 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 bloodstream, 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. Outward signs of this condition are redness and the appearance of cyanotic spots on the skin. For the purpose, 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.

and stomach

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 is the necrosis (necrosis) of a section of the organ wall and the formation of an ulcerative 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. from the wall of the vessel, they can, bypassing the heart, penetrate 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 peripheral blood changes and its clotting increases, gingival bleeding may 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, An increase in the viscosity properties of blood activates the compensatory reaction of the vessels as a consequence, 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 is reliable evidence of the presence of Vakez disease.

With polycythemia, the morphology of erythrocytes is preserved, that is, they do not change their normal shape and size. However, with the development of anemia 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 red blood cells can be restored by diluting the blood by adding a liquid part to it (high-molecular 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 with polycythemia.

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, it is very rarely carried out 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 have been provoked. The baby's body begins to synthesize a large number of red blood cells to correct hypoxia.

In addition to respiratory conditions, newborns may 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 polyferation 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 weight loss, 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!