Polycythemia (erythremia, Wakez's disease): causes and development, manifestations, treatment, prognosis. How is blood erythremia treated and the symptoms of Wakez disease Causes of Wakez disease

Polycythemia is a disease that can be determined by looking only at a person's face. And if you still hold diagnostic examination, then there will be no doubt at all. In the medical literature, you can find other names for this pathology: erythremia, Wakez's disease. Regardless of the term chosen, the disease carries a serious threat to human life. In this article, we will talk in more detail about the mechanism of its occurrence, primary symptoms, stages, and proposed treatments.

general information

Polycythemia vera is a myeloproliferative blood cancer in which it produces red blood cells in excess quantities. To a lesser extent, there is an increase in other enzymatic elements, namely leukocytes and platelets.

Red blood cells (otherwise erythrocytes) saturate all cells with oxygen human body, delivering it from the lungs to the systems internal organs. They are also responsible for removing carbon dioxide from the tissues and transporting it to the lungs for later exhalation.

Red blood cells are continuously produced in the bone marrow. It is a collection of spongy tissues, localized inside the bones and is responsible for the process of hematopoiesis.

Leukocytes are white blood cells that help fight various infections. Platelets are fragments that are activated when the integrity of blood vessels is violated. They have the ability to stick together with each other and clog the hole, thereby stopping the bleeding.

Polycythemia vera is characterized by excessive production of red blood cells.

Disease prevalence

This pathology is usually diagnosed in adult patients, but can occur in adolescents and children. For a long time, the disease may not make itself felt, that is, it may be asymptomatic. According to the research, average age patients varies from 60 to approximately 79 years. Young people get sick much less often, but the disease is much more severe for them. The representatives of the stronger sex, according to statistics, are several times more likely to be diagnosed with polycythemia.

Pathogenesis

Most of the health problems associated with this disease are due to the continuous increase in the number of red blood cells. As a result, the blood becomes excessively thick.

On the other hand, its increased viscosity provokes the formation of clots (thrombi). They can interfere with normal blood flow through the arteries and veins. This situation often causes strokes and heart attacks. The thing is that thick blood flows several times slower through the vessels. The heart has to work harder to literally push it through.

The slowdown in blood flow does not allow the internal organs to receive the necessary amount of oxygen. This entails the development of heart failure, headaches, angina pectoris, weakness and other health problems that are not recommended to be ignored.

Disease classification

• I. Initial stage.

1. Lasts 5 years or more.
2. The spleen is of normal size.
3. Blood tests show a moderate increase in the number of red blood cells.
4. Complications are extremely rare.

• II A. Polycythemic stage.

1. Duration from 5 to approximately 15 years.
2. There is an increase in some organs (spleen, liver), bleeding and thrombosis.
3. There are no sites in the spleen itself.
4. Bleeding can cause iron deficiency in the body.
5. In the blood test, there is a persistent increase in erythrocytes, leukocytes and platelets.

• II B. Polycythemic stage with myeloid metaplasia of the spleen.

1. Analyzes show an increased content of all blood cells, except for lymphocytes.
2. There is a tumor process in the spleen.
3. In the clinical picture appears exhaustion, thrombosis, bleeding.
4. In the bone marrow there is a gradual formation of scars.

• III. anemic stage.

1. In the blood, there is a sharp decrease in erythrocytes, platelets and leukocytes.
2. There is a pronounced increase in the size of the spleen and liver.
3. This stage usually develops 20 years after the diagnosis is confirmed.
4. The disease can transform into acute or chronic leukemia.

Causes of the disease

Unfortunately, at present, experts cannot say which factors lead to the development of such a disease as polycythemia vera.

Most tend to the virus-genetic theory. According to her, special viruses (there are about 15 of them in total) are introduced into the human body and under the influence of some factors that adversely affect immune defense, penetrate into the cells of the bone marrow and lymph nodes. Then, instead of maturation, these cells begin to rapidly divide and multiply, forming more and more new fragments.

On the other hand, the cause of polycythemia may be hidden in hereditary predisposition. Scientists have proven that close relatives of a sick person, as well as people with violations of the structure of chromosomes, are more susceptible to this disease.

Factors predisposing to the onset of the disease

• X-ray exposure, ionizing radiation.
• Intestinal infections.
• Viruses.
• Tuberculosis.
• Surgical interventions.
• Frequent stress.
• Long-term use of certain groups of drugs.

Clinical picture

Starting from the second stage of the development of the disease, literally all systems of internal organs are involved in the pathological process. Below we list the subjective sensations of the patient.

• Weakness and haunting tiredness.
• Increased sweating.
• Noticeable decrease in performance.
• Severe headaches.
• Memory deterioration.

Polycythemia vera may also be accompanied by the following symptoms. In each case, their severity varies.

Diagnostics

First of all, the doctor collects a complete anamnesis. He can ask a number of clarifying questions: when exactly the malaise / shortness of breath / painful discomfort appeared, etc. It is equally important to determine the presence of chronic ailments, bad habits, possible contact with toxic substances.

Then a physical examination is performed. The specialist determines the color skin. By palpation and percussion reveals an increase in the spleen or liver.

Blood tests are required to confirm the disease. If the patient has this pathology, the test results may be as follows:

• An increase in the number of red blood cells.
• Elevated hematocrit (percentage of red blood cells).
• High levels of hemoglobin.
• Low level of erythropoietin. This hormone is responsible for stimulating the bone marrow to produce new red blood cells.

Diagnosis also includes brain aspiration and biopsy. The first version of the study involves the collection of the liquid part of the brain, and a biopsy of the solid component.

Polycythemia disease is confirmed by gene mutation tests.

What should be the treatment?

It is not possible to completely overcome a disease such as polycythemia vera. That is why therapy focuses solely on reducing clinical manifestations and reducing thrombotic complications.

Patients are given bloodletting first. This procedure involves the removal of a small amount of blood (from 200 to approximately 400 ml) with therapeutic purpose. It is necessary to normalize the quantitative parameters of blood and reduce its viscosity.

Patients are usually prescribed "Aspirin" to reduce the risk of developing various kinds of thrombotic complications.

Chemotherapy is used to maintain a normal hematocrit when there is severe itching or increased thrombocytosis.

Bone marrow transplantation in this disease is extremely rare, since this pathology, in the case of adequate therapy, is not deadly.

It should be noted that the specific treatment regimen in each case is selected individually. The above therapy is for informational purposes only. It is not recommended to try to cope with this disease on your own.

Possible Complications

This disease is quite serious, so do not neglect its treatment. Otherwise, the likelihood of unpleasant complications increases. These include the following:

Forecast

Wakez disease is rare disease. Symptoms that appear in the early stages of its development should be the reason for immediate examination and subsequent therapy. In the absence of adequate treatment, if the disease was not diagnosed in a timely manner, death occurs. main reason lethal outcome most often there are vascular complications or transformation of the disease into chronic leukemia. However, competent therapy and strict adherence to all the recommendations of the doctor can significantly extend the life of the patient (by 15-20 years).

We hope that all the information presented in the article will be really useful for you. Be healthy!

Polycythemia is a neoplastic disease of a permanent nature associated with the circulatory system. Appears in the conspicuous. This is followed by a decrease in blood flow activity, which in turn leads to the appearance of blood clots and hypoxia, in this process the erythroblastic germ is most damaged, and all because of an increase in blood viscosity. The name Wakez Osler's disease comes from the name of the scientist who first described it.

What it is

Wakez disease, what is it? Most often, this disease is subject to the elderly, but often it is men who suffer. But it should not be ruled out that young people cannot get sick, and Osler's disease itself is much more difficult for the unlived than for elderly people. This disease has a genetic subcortex, and the causes of Wakez's disease have not been established even at the present time, although it was known back in 1892, but there are still options for treating Wakez's disease.

Symptoms

In the early stages of the disease visible symptoms Wakez Osler are absent, so the patient is usually unaware of the presence of polycythemia in combination with the fact that the disease proceeds rather slowly. But when the disease intensifies, the first symptoms of Wakez's disease appear, namely, this is expressed in the appearance of migraine and dizziness, in the presence of a strange sensation inside the skull.

In the progressive stage of the disease, symptoms such as:

• Itching of the epidermis. Often, itching sensations increase after skin contact with water, which is caused by the release of histamine, prostaglandin and serotonin into the blood;
• , and a change in the shade of the epidermis. The epidermis in the area of ​​​​the physiognomy, hands and neck takes on a reddish tint. Also, upon careful study, you can notice a change in the color of the mucous membranes to dark red with blue. Because of varicose veins veins on the neck swollen veins are clearly visible;
• , do not forget that bleeding gums are a symptom of many more serious illnesses;
• Enlargement of the liver and spleen;
• Colic in legs and joints.

There are several other symptoms of Wakez, which are less common, but can also indicate Wakez's disease: rapid asthenia, blurred vision, hearing damage, frequent mood changes, lethargy. Polycythemia is also associated with uncontrolled weight loss, repeated colds.

Diagnostics

The conclusion is set by the doctor, after viewing a detailed picture, namely the diagnosis of the bone marrow. There are further points in determining the presence of Wakez disease.

Clinical symptoms of Osler:

• Sudden and uncontrolled weight loss;
• Evaluation of the color of the epidermis and mucous membranes of the patient.
• Predisposition to thrombosis and azotemia of the spleen and liver.
• Dizziness.

Result special analysis may be an indicator of polycythemia, if sharply.

What changes occur in the blood

An increase in the level of red blood cells and hemoglobin, and a decrease in the color index, which takes on a value of about one. Due to the increase in the level of erythrocytes, the blood volume can double, and the hematocrit changes in the range of up to 65%. Neutrophils increase up to 80%, and grows 1.5-2 times.

Treatment

The main methods of treatment for Wakez disease are bloodletting and chemotherapy.

And it brings the ratio of erythrocytes and hemoglobin to the usual value, by reducing the range of blood. The volume of phlebotomy is 500 milliliters per procedure. Such procedures should take place under the supervision of a hematologist, and with an interval of 2 days, and so on until the hemoglobin index returns to normal 140-150 and lowers its level to 45%. This method is useful in that it reduces irritation of the epidermis and excites the bone marrow, stabilizing its work. Phlebotomy is often used in conjunction with chemotherapy.

Chemotherapy is used to hibernate erythrocytes and platelets, preventing their regeneration. For this procedure, some pharmacological preparations belonging to several groups are used: imifos, myelbromol, myelosan, chlorambucil. Each patient will have an original treatment regimen, which will be selected by the attending physician.

Monitoring

If Wakez's disease is indeed diagnosed, then most likely everything will end positively, because often polycythemia occurs benignly. The patient can last a very long time, especially if used modern methods treatment.

The starting point of the disease is often cirrhosis of the liver, or myeloid leukemia. Therefore, it is always necessary to be examined at least once every six months by a doctor in order to detect Wakez's disease at an early stage.

If a person has high hemoglobin and overestimated erythrocyte counts in the blood, then this by no means can say that he is healthy. Unfortunately, these indicators are the first signs of Wakez's disease. There are also a number of other symptoms that you should definitely pay attention to and immediately consult a specialist. In addition, it will be useful to find out other information about the pathology, which will help to identify this disease in a timely manner and begin its treatment.

general information

Wakez's disease (also called true or primary polycythemia) is a chronic neoplastic pathology that develops in the human circulatory system. As a rule, it is possible to identify this disease thanks to diagnostic studies, during which it turns out that the level of red blood cells in the patient's blood is too high. However, at the same time, patients do not experience any damage to internal organs, in which such indicators are considered the norm.

Also, with the development of Wakez's disease, the structure of the blood becomes more viscous and thick. Against this background, there is a strong slowdown in blood flow, which is why the formation of blood clots is possible. This condition can lead to hypoxia.

If we talk about the history of Wakez's disease, then for the first time it was described back in 1892. Then one scientist discovered the relationship between some indicators of patients and the development of erythremia. Subsequently, the disease was named after the specialist himself. Therefore, in medical reference books, this pathology is more often found under the name Wakez Osler's disease.

As far as who is most prone to this disease, then with the greatest care to their health should be treated by people of advanced age. Most often, men suffer from polycythemia. Children and middle-aged people are much less likely to experience this disease. However, if the younger generation shows the first signs of the disease, then in this case, Wakez's disease is much more severe.

What happens to the body when sick

During polycythemia vera, the volume of red blood cells in a person's blood begins to increase greatly. Every day the patient's condition worsens. Against this background, leukocytosis and thrombocytosis begin to develop. Such processes of the pathological type increase the viscosity of the blood, which leads to a significant increase in its volume. The movement of vital fluid is greatly slowed down. The tissues of the human body cease to receive the required amount of oxygen.

Considering the pathogenesis of Wakez's disease, it is worth considering that in some situations, at the initial stage of the development of the disease, the body begins to independently use compensatory mechanisms. However, its protective functions are not enough for a long time, even if a person is famous for his excellent immunity.

If the patient does not receive appropriate treatment, then, according to the pathophysiology, Wakez's disease will begin to manifest itself more aggressively. After some time, the patient will have serious problems with high blood pressure. The spleen and liver may be greatly enlarged. In an advanced degree, this pathology leads to disruption of the kidneys.

Against the background of all this, the blood supply to the human heart and brain slows down significantly, and the limbs cease to function normally.

If a person does not take any measures for treatment, then the disease can even develop into malignant polycythemia vera. Wakez's disease is very dangerous, but this does not mean that patients should give up on themselves.

Reasons for the development of polycythemia

It is difficult to say about the specific factors that lead to the development of pathology. This disease is still being studied by medical specialists. However, the etiology and pathogenesis of Wakez disease contain information that the disease develops against the background of mutational changes. They occur in stem cells located in the bone marrow. Accordingly, we are talking about a genetic pathology.

Very often, several relatives have the same form of erythremia at once. Wakez's disease also develops against the background of increased erythropoiesis. As a rule, absolute erythrocytosis leads to it.

Also, disorders associated with blood clotting can provoke a similar condition. However, experts admit other causes of the pathology.

Is this a cancer

Many patients, having heard that the pathology belongs to the category of leukemia, begin to panic and associate it with blood cancer. However, this assumption is not entirely correct. First of all, it is worth mentioning the malignant and benign nature of such an education. As a rule, when it comes to cancer, in this case, doctors talk about a tumor that consists of epithelial tissues. However, polycythemia (Wakez's disease) is a formation consisting of hematopoietic tissue. Nevertheless, this disease can still be attributed to malignant tumors.

However, it should be borne in mind that such a pathology is characterized by high differentiation of cells. In this case, there is a strong difference from cancer. The fact is that this disease can be in the chronic stage for a very long time. This means that the tumor is classified as benign. If the treatment of Wakez's disease is done in a timely manner, then there is every chance for a full recovery.

If we allow the last stage of the development of the disease, then in this case we will talk about the real malignant tumor. Therefore, it is better not to take risks.

Symptoms

At the initial stage of development, pathology is quite difficult to identify. Even if patients experience any discomfort, the least likely to be associated with such a deteriorating state of health is polycythemia. However, with the progression of the disease, the clinical picture becomes more pronounced, and doctors note the obvious features of the manifestation of Wakez's disease.

With the development of polycythemia:

• Subcutaneous veins expand and swell. The skin may take on a reddish or bluish tinge. This is the main sign that the outflow of blood has slowed down significantly and the vessels began to overflow with fluid.
• There is a violation of blood microcirculation in the brain. At the same time, patients complain of severe headaches, weakness, dizziness. It becomes very difficult for them to concentrate. Many report problems with vision and hearing.
• Appears arterial hypertension.
• The itching of the skin begins. It is noteworthy that itchy discomfort appears in the process of skin contact with warm water. This means that while taking a shower or bath, a person experiences severe discomfort.
• Pain in the extremities is noted.
• There are pains in the joints, reminiscent of gout.
• Ulcerative pathologies develop in the stomach and duodenum.

In addition, the symptoms of Wakez disease often include the formation of blood clots. Therefore, do not turn a blind eye to such manifestations.

Stages of development of the disease

Since polycythemia in the first stages practically does not manifest itself in any way, all the symptoms described above occur only with a more serious development of the pathology. This disease is usually divided into three stages.

At the first stage, doctors note the satisfactory state of health of the patient. Erythremia (Wakez's disease) is characterized by the absence of pronounced symptoms. This stage can take up to 5 years. During this time, neither the doctors nor the patient himself can even guess that he is suffering from this dangerous disease.

After this, the second stage begins, which is characterized by extended clinical manifestations. It is divided into 2 categories. In the first case, doctors do not note metaplasia of the spleen. At the same time, some patients pay attention to the appearance of some symptoms characteristic of erythremia. This stage can develop over 15 years.

There is also a second category of the 2nd stage of the development of pathology. In this case, the development of metaplasia of the spleen occurs. Symptoms begin to appear more clearly. Most of all, doctors pay attention to a significant increase in the size of the liver and spleen.

This is followed by stage 3. This stage in the development of pathology is also called terminal. Patients show almost all the symptoms that they suffer from a malignant process in the body. At the same time, patients often claim that almost their entire body hurts. This is due to the fact that during the thermal stage of pathology, the cells of the human body cease to differentiate, which creates a substrate for leukemia. In this case, we are no longer talking about chronic erythremia.

In the process of the terminal stage, a very serious condition of patients is observed. Some people have ruptures of the spleen, infectious and inflammatory processes occur in the body, and hemorrhagic syndrome may develop. Also, certain groups of patients show immunodeficiency. Treatment of the disease is much more complicated. If the pathology has been brought to the final stage, then there is a high risk that it will end in death.

Is it possible to live with illness

You need to understand that life expectancy with a diagnosis can be up to 20 years. If a patient has a disease at the age of 60-80, then he may well not take any measures and continue a normal existence.

However, it all depends on the individual and the state of his body. In some situations, even in the early stages of polycythemia, the symptoms become pronounced. Because of this, it is much more difficult for a person not to notice the disease.

Diagnostics

This pathology is quite easily detected if the doctor conducts a general and biochemical analysis blood. Wakez disease is detected due to an increased volume of red blood cells and hemoglobin. In this case, hematocrit values ​​can reach up to 65% or more. Among the additional indicators, it is worth noting the increased concentration of uric acid. Also, with Wakez disease, a blood test will show an increased volume of young red blood cells.

However, in some situations, experts doubt the correctness of the diagnosis. To make sure that a person really suffers from polycythemia, it is necessary to perform a puncture of the brain. If there is a suspicion that the disease has been transmitted genetically, an appropriate blood test will be required.

Additionally, the doctor needs to clarify how severe the degree of damage to human organs. To this end, patients are referred to ultrasound, ECG and other diagnostic procedures.

Treatment

There are several options that help alleviate the condition of a sick person. First of all, the so-called bloodletting is performed. Also, some experts prefer to resort to selective removal of certain red blood cells from the blood. This procedure is performed using very expensive equipment. Cytostatic agents and interferon alpha may also be used.

If speak about drug treatment, then in this case the most commonly used:

• "Allopurinol". This drug helps to significantly reduce the amount of uric acid in the blood. This improves kidney function and eliminates strong pain syndrome in human limbs.
• Antiplatelet agents and anticoagulants. These drugs are necessary in order to normalize the processes of blood microcirculation. This will help prevent the formation of blood clots.
• Antihistamines. They help a person cope with itchy skin.

Traditional medicine and proper nutrition

First of all, it is worth noting that in order to get rid of polycythemia, it is very important to eat right and make sure that a person does not overload himself physically. If we are talking about the very beginning of the development of pathology, the patient is assigned table number 15. However, there are some reservations. First of all, the patient should not eat those foods that can increase blood flow and hematopoiesis, so you need to stop eating the liver. It is worth consuming dairy and vegetable products.

If the patient's condition is characterized by the second stage of the pathology, then table number 6 is assigned to him. A similar diet is recommended for gout. In this case, meat and fish dishes must be completely excluded from your diet. It is also necessary to abandon legumes and sorrel.

After the patient is discharged from the hospital, he must carefully monitor his health and follow all the doctor's recommendations.

If speak about folk treatment, then almost all patients are interested in this issue. You need to understand that this pathology develops for many years, respectively, it will be problematic to choose the appropriate treatment with the help of herbs. Even if the symptoms of the pathology subsided, this does not mean that the patient is completely cured. He may still face exacerbations of the disease. Unfortunately, with the help of herbs, you can only relieve pain or dull it. However, there is no question of a complete cure in this case.

First of all, you need to talk with your doctor, and only then think about folk ways treatment. If the physician agrees to additional methods therapy, it must be remembered that we are talking about a blood disease.

On the network you can find a huge number of recipes that will help increase the level of hemoglobin. However, this does not mean that these recommendations are appropriate in the fight against polycythemia. Erythremia (Wakez's disease) is not so easy to treat natural remedies. Although the doctors do not mind traditional medicine, if we are talking about polycythemia, then almost everyone agrees that in this case, pick up a complex medicinal herbs impossible. The course of the pathology is reflected in the work of the bone marrow and affects circulatory system. It is necessary to objectively evaluate all the possibilities of natural preparations.

However, to improve their condition, some resort to prescriptions for blood thinning. For example, you can brew a spoonful of sweet clover grass in a glass of boiling water. After the mixture has cooled, it must be filtered through cheesecloth. The finished medicine is taken three times a day, approximately 80-100 ml. The course of such treatment should not exceed one month. After that, you need to take a break.

Many also use dry or fresh cranberries. Two tablespoons of a natural product are poured with a glass of boiling water. After that, the container must be covered and wait 20-30 minutes until the broth is infused. After that, the sour juice can be diluted with honey or regular sugar. The main advantage of this method is that you can drink cranberries in any quantity if the patient does not have an allergy to it.

Forecast

If we are talking about this pathology, then you should not immediately draw an analogy with cancerous tumors. Wekez's disease responds well to treatment. However, therapeutic measures should not be taken at the last stage of the disease. Good news is that the disease develops for a very long time. During this time, a person is guaranteed at least once to be examined by a doctor who will pay attention to blood tests.

However, in the absence of treatment, the patient is likely to develop complications. For example, some patients suffer from ischemia, pulmonary embolism, cirrhosis, and many other incurable pathologies that can lead to death. Therefore, one should not be afraid of visiting a doctor, since a disease detected in time is much easier and faster to cure.

Although there is a high probability of a complete cure, in some situations this pathology returns.

Information in conclusion

There is also relative erythremia. This pathology has nothing to do with true polycythemia, so do not confuse these two ailments. The relative form of the disease most often develops against the background of somatic manifestations, according to a completely different pattern. This pathology is much easier to treat.

However healthy eating important for any person, regardless of what he is sick with. Therefore, it is important to keep track of the products that fall on the dining table and spend as much time as possible in the fresh air. Such universal preventive measures will help protect against a huge number of diseases.

Erythremia (ER) is a myeloproliferative disease,

Imic, benign current leukemia, in which there is

increased formation of erythrocytes, as well as neutrophilic leukocytes

ov and platelets. The source of tumor growth is the progenitor cell

Tsa myelopoiesis.

The incidence of erythremia is about 0.6 per 10,000 population. Both men and women are equally often ill. Erythremia is a disease of the elderly: the average age of patients is 55-60 years, but the disease is possible at any age.

Etiology. The reasons for the development of the disease are unknown.

Pathogenesis. Erythremia is based on tumor clonal proliferation of all three hematopoietic lineages - red, granulocytic and megakaryocytic, however, the growth of the red lineage dominates. In this regard, the main substrate of the tumor are erythrocytes maturing in excess. Foci of myeloid hematopoiesis appear in the spleen and liver (which is never normal). An increased number of erythrocytes and platelets in the peripheral blood reduces the speed of blood flow, increases the viscosity and coagulability of the blood, which causes a number of clinical symptoms.

Classification. The stage of the course of the disease, the involvement of the spleen in the pathological process and the subsequent transformation of erythremia into other diseases of the blood system are taken into account.

Stage I - initial: hemoglobin content at the upper limit of normal, a slight increase in the mass of circulating red blood cells, the spleen is slightly enlarged (due to overflow with blood) or without changes. Blood pressure is normal or slightly elevated, there is focal bone marrow hyperplasia in the trepanate from the ilium. The duration of stage I can exceed 5 years.

Stage II - expanded: phase A - without myeloid metaplasia of the spleen (a simple plethora variant without splenomegaly). Total three-line hyperplasia of the bone marrow. Absence of extramedullary hemopoiesis; phase B - with myeloid metaplasia of the spleen. Major myeloproliferative syndrome: pancytosis in the peripheral blood, panmyelosis in the bone marrow with or without focal myelofibrosis, myeloid metaplasia of the spleen with or without fibrosis.

Stage III - terminal: degeneration of a benign tumor into a malignant one (myelofibrosis with anemia, chronic myelogenous leukemia, acute leukemia). Myelofibrosis develops in almost all patients for more than 10-15 years; it reflects the natural evolution of the disease. A sign of myelofibrosis is cytopenia (anemia, thrombocytopenia, less often - leukopenia). The development of chronic myeloid leukemia is manifested by an increase in leukocytosis, an increase (or appearance) in the peripheral blood of myelocytes, promyelocytes, and also by the detection of the Ph chromosome in blood and bone marrow cells.

Acute leukemia usually develops in patients treated with cytostatics and radioactive phosphorus.

Anemia in patients with erythremia may be associated with frequent bleeding, increased deposition of erythrocytes, as well as their hemolysis.

clinical picture. Erythremia is manifested by two large syndromes.

Plethoric syndrome due to an increased content of erythrocytes, as well as leukocytes and platelets (plethora - plethora). This syndrome is caused by: 1) the appearance of subjective symptoms; 2) violations of cardio-vascular system; 3) changes in laboratory parameters.

1. Subjective symptoms of plethoric syndrome include headaches, dizziness, blurred vision, angina pectoris, pruritus, erythromelalgia (sudden onset of hyperemia with

nuanced skin tone of the fingers, accompanied by sharp pains and burning), sensations of numbness and chilliness of the extremities are possible.

2. Disorders of the cardiovascular system are manifested in a change in the color of the skin and visible mucous membranes according to the type of erythrocynoses, features of the color of the mucous membrane at the point of transition of the soft palate to the hard palate (Kuperman's symptom), hypertension, the development of thrombosis, less often bleeding. In addition to thrombosis, swelling of the legs and erythromelalgia are possible. Circulatory disorders in the arterial system can lead to severe complications: acute infarction myocardial infarction, stroke, visual impairment, renal artery thrombosis.

3. Changes in laboratory parameters: an increase in the content of hemoglobin and erythrocytes, an increase in hematocrit and blood viscosity, moderate leukocytosis with a shift leukocyte formula to the left, thrombocytosis, a sharp slowdown in ESR.

Myeloproliferative Syndrome due to hyperplasia of all three hematopoietic sprouts in the bone marrow and extramedullary. It includes: 1) subjective symptoms, 2) splenomegaly and (or) hepatomegaly, 3) changes in laboratory parameters.

1. Subjective symptoms: weakness, sweating, fever
body tours, bone pain, heaviness or pain in the left hypochondrium (due to

splenomegaly).

2. Splenomegaly is explained not only by myeloid metaplasia of the organ (the appearance of foci of extramedullary hematopoiesis), but also by blood stasis. Rarely, the liver is enlarged.

3. Among the laboratory parameters, deviations from the physiological norm in the peripheral blood have the greatest diagnostic value: pancytosis, more often with a shift of the leukocyte formula to the left; trephine biopsy reveals three-line hyperplasia of the bone marrow, and foci of myeloid metaplasia of the organ are found in the punctate of the spleen.

Different severity of syndromes at different stages of the disease causes extreme variability clinical picture. It is possible to observe patients with undoubted erythremia, almost no complaints and fully able-bodied, and patients with severe damage to internal organs who need therapy and have lost their ability to work.

At stage I of the diagnostic search in the initial stage of the disease, patients may not present any complaints. As the disease progresses, complaints are associated with the presence and severity of plethora and myeloproliferative process. The most frequent complaints are of a "plethoric" nature, due to increased blood filling of the vessels and functional neurovascular disorders (headaches, erythromelalgia, visual impairment, etc.). All these symptoms may be associated with other diseases, which must be clarified during further examination of the patient.

Complaints caused by the presence of myeloproliferative syndrome (sweating, heaviness in the left hypochondrium, bone pain, fever) are also nonspecific for erythremia. Skin itching is quite characteristic, which appears after taking water procedures. This symptom is observed in 55% of patients in the advanced stage and is explained by hyperproduction of basophils and histaminemia. The nature of urticaria observed in 5-7% of patients is similar.

The listed symptoms are important for determining the stage of erythremia: they usually indicate the transition of the disease to a developed

or the terminal stage with the development of myelofibrosis as the most common outcome of erythremia.

In the anamnesis of patients there may be such complications of the disease as strokes, myocardial infarctions. Sometimes the disease debuts precisely with these complications, and the true cause of their development - erythremia - is detected when examining a patient for a stroke or myocardial infarction.

Indications of previous treatment with radioactive phosphorus, cytostatics, or bloodletting may suggest the presence of some kind of neoplastic blood disease. A decrease in the symptoms of plethoric syndrome during treatment with these agents suggests erythremia.

At the II stage of the diagnostic search, it is possible to identify distinct symptoms only in the II (expanded) stage of the disease. Mostly signs of plethoric syndrome are found: erythrocyanosis, injected vessels of the conjunctiva (“rabbit eyes”), a distinct color border at the transition point of the hard palate to the soft palate. It is possible to identify symptoms of erythromelalgia: swelling of the fingertips, feet, lower third of the lower leg, accompanied by local hyperemia and a sharp burning sensation.

In the study of the cardiovascular system, hypertension and an increase in the left ventricle are diagnosed, in the advanced stage of the disease - "variegated legs" (discoloration of the skin of the legs, mainly their distal part) in the form of pigmentation areas of varying intensity, due to impaired venous circulation.

On palpation of the abdomen, an enlarged spleen can be detected, which is one of the characteristic signs of the disease. Enlargement of the spleen may be due to: 1) increased deposition of blood elements; 2) "working" hypertrophy due to an increase in its sequestering function; 3) extramedullary hematopoiesis (myeloid metaplasia with a predominance of erythropoiesis). These reasons are often combined. Liver enlargement is due to similar causes, as well as the development of fibrosis and nonspecific reactive hepatitis. It should be borne in mind that hepatomegaly can be observed in a malignant liver tumor with the development of secondary erythrocytosis.

Complications of erythremia in the form of thrombosis of cerebral vessels are expressed by a number of focal symptoms detected during the study.

However, even at stage II, it is impossible to make a definitive diagnosis of erythremia, since many of its symptoms can be associated with symptomatic erythrocytosis. In addition, symptoms such as hypertension, splenomegaly, and hepatomegaly are characteristic of a wide variety of diseases.

Concerning III the stage of diagnostic search is of decisive importance, as it allows: a) to make a final diagnosis; b) clarify the stage of erythremia; c) identify complications; d) to control the treatment.

Peripheral blood test detects erythrocytosis, an increase in hemoglobin and hematocrit, which, however, also occurs with symptomatic erythrocytosis. Diagnostic value has an increase in hemoglobin levels in combination with erythrocytosis, leukocytosis and thrombocytosis. When examining the leukocyte formula, a shift to the left to immature forms of granulocytes is detected. If changes in peripheral blood are insignificant or data are inconclusive (for example, erythrocytosis is not combined with thrombocytosis), then it is necessary to conduct bone marrow examination (trepanobiopsy). Presence in trepanate total-442

A layer of three-growth hyperplasia of the bone marrow with a predominance of Hb ix elements of erythropoiesis, the replacement of adipose tissue with a red germ of the bone marrow make it possible to make a final diagnosis. The expansion of the "bridgehead" of hematopoiesis is also detected using radionuclide bone scan with 32 R. Histochemical study reveals increased activity of alkaline phosphatase of neutrophils.

Complications. The course of erythremia is complicated by: 1) vascular thrombosis (cerebral, coronary, peripheral arteries); 2) hemorrhagic syndrome: bleeding after minor surgical interventions (tooth extraction), from the vessels of the digestive tract, hemorrhoids, which is due to poor retraction of the blood clot due to changes in the functional properties of platelets; 3) endogenous uricemia and uricosuria (due to increased cell death at nuclear prestages of their maturation), which is manifested by symptoms urolithiasis and gouty arthritis.

The outcomes of the disease are the situations indicated in the III stage of the course of the disease (myelofibrosis, chronic myeloid leukemia, acute leukemia, anemia).

Diagnostics. Erythremia can be suspected in individuals with persistent erythrocytosis associated with neutrophilic leukocytosis, thrombocytosis, in the absence of diseases (or conditions) that could cause erythrocytosis.

diagnostic criteria erythremia (in the advanced stage) are:

Increase in the mass of circulating red blood cells.

Normal saturation of arterial blood with oxygen (more than 92%).

Leukocytosis more than 12 10 9 /l (in the absence of obvious reasons for the appearance of leukocytosis).

Thrombocytosis more than 400-10 9 /l.

An increase in the content of alkaline phosphatase of neutrophils (in the absence of infection).

Increase in unsaturated vitamin B 12-binding capacity of blood serum.

The diagnosis of ER is confirmed by the presence of three category A signs or two category A signs and one category B sign.

Difficulties in making a diagnosis are due to the development of the so-called symptomatic erythrocytosis in a number of diseases. Allocate absolute and relative erythrocytosis. With absolute erythrocytosis, an increase in the mass of circulating erythrocytes and increased erythropoiesis are noted. Relative erythrocytosis is characterized by a decrease in the volume of circulating plasma and a normal mass of circulating erythrocytes. Relative erythrocytosis is often detected in men suffering from hypertension, obesity, neurasthenia, taking diuretics. Secondary absolute erythrocytosis develops in smokers, it is due to an increase in the content of carbon monoxide in the blood.

Reasons for the development of symptomatic erythrocytosis: 1) generalized tissue hypoxia (pulmonary pathology, heart disease, hemoglobinopathies, obesity, etc.); 2) paraneoplastic reactions (noch tumors, tumors of the adrenal cortex and medulla, pituitary gland, ovaries, vascular tumors, tumors of other organs); 3) renal ischemia

(stenosis renal artery, hydronephrosis, polycystic and other kidney anomalies); 4) unknown causes (CNS disease, portal hypertension).

Relative erythrocytosis observed in exicosis (dehydration due to diarrhea, vomiting, excessive sweating, etc.). Differential diagnosis is based on taking into account the entire clinical picture. In difficult cases, it is necessary to investigate the content of erythropoietin in the blood; with erythremia, it does not increase.

Formulation of a detailed clinical diagnosis includes information about-1) the stage of the disease; 2) the presence of complications; 3) phase of the process (exacerbation or remission); 4) the presence of pronounced syndromes (portal hypertension, hypertension, etc.).

Treatment. The whole complex of therapeutic measures for ER is as follows.

In the advanced stage of the disease, in the presence of plethoric syndrome, but without leuko- and thrombocytosis, bloodletting is used as an independent method of therapy, while it is necessary to achieve a decrease in hematocrit to normal values ​​(less than 45 %). Take 400-500 ml of blood every other day (in a hospital) or 2 days later (in a clinic). For the prevention of thrombosis (developing as a result of bloodletting, as well as a complication of erythremia), acetylsalicylic acid is prescribed at a dose of 0.5-1 g / day on the eve and on the day of bloodletting, and then within 1-2 weeks after the end of bloodletting. In addition to acetylsalicylic acid, other antiplatelet agents are also prescribed - ticlide, fluoride, pentoxifylline. Before bloodletting to prevent thromboembolism pulmonary artery it is advisable to inject 5000 IU of heparin intravenously (through a Dufo needle), as well as 5000 IU of heparin under the skin of the abdomen 2 times a day for several days after bloodletting. In case of poor tolerability of bloodletting, observed in persons with severe atherosclerosis of cerebral vessels, exfusion is limited to 350 ml (2 times a week). When bleeding, it is necessary to reduce hemoglobin to 150 g / l.

If bloodletting is not effective enough, as well as in forms of the disease that occur with pancytosis and splenomegaly, cytostatic therapy is prescribed. The age of patients over 55 years expands the indications for the use of cytostatics. Indirect indications for cytostatic therapy are other signs of myeloproliferative syndrome (itching), as well as the severity of the disease, visceral vascular complications (stroke, myocardial infarction), exhaustion.

Contraindications to cytostatic therapy: young age of patients, refractoriness to treatment at previous stages, as well as excessively active cytostatic therapy in the past due to fear of the transition of the disease to the phase of anemia. The effect of cytostatic therapy should be assessed 3 months after the end of treatment; this is explained by the fact that the erythrocytes produced before treatment live on average for about 2-3 months. The decrease in the number of leukocytes and platelets occurs much earlier, according to their life span. The criterion for the effectiveness of cytostatic therapy is the achievement of hematological remission (complete, when all blood counts are normal, or partial, in which the number of erythrocytes, leukocytes and / or platelets remains slightly elevated).

Of the cytostatic drugs at the first stage, hydroxyurea (hydrea) is usually prescribed at a dose of 30-50 mg / (kg per day) (2-3 capsules per

day). During treatment, it is necessary to control the number of leukocytes. Hydrea is combined with a-interferon at a dose of 3-5 million IU subcutaneously 3-7 times a week for a long time (at least a year), which allows stopping thrombocytosis, plethora, and pruritus.

Anagrelide is used for hyperthrombocytosis.

The outcomes of erythremia (myelofibrosis, acute leukemia, chronic myelogenous leukemia) are affected according to the principles of treatment of these diseases: in myelofibrosis, anabolic steroids, nitostatics and transfusions are used erythrocyte mass; in acute leukemia, polychemotherapy is indicated, in chronic myeloid leukemia - cytostatic drugs.

Symptomatic therapy with attacks of erythromelalgia, it is carried out with the help of antiplatelet agents, non-steroidal anti-inflammatory drugs ( acetylsalicylic acid, indomethacin). Arterial hypertension, angina attacks are eliminated in accordance with the rules for the treatment of these conditions.

When erythremia is complicated by vascular thrombosis, anticoagulant and antiplatelet therapy is used.

Patients with erythremia are put on a dispensary record with the frequency of visiting a doctor and the appointment of peripheral blood tests 1 time in 3 months.

Forecast. With an uncomplicated course of erythremia, life expectancy can reach 15-20 years (complications arise in the future). If complications from the cardiovascular system develop early enough or the disease progresses, life expectancy is reduced. Timely initiated therapy prolongs life expectancy, although this is not observed in all cases.

Prevention. There are no radical measures to prevent the disease, and therefore we can only talk about secondary prevention, which consists in dynamic monitoring of patients and anti-relapse therapy.

Causes of polycythemia

The exact cause of Wakez disease has not yet been established. However, there are a number of factors that increase the risk of developing it. These include:

• genetic predisposition. Blood erythremia disease is often found in people with genetic pathologies such as Down's syndrome, Klinefelter's syndrome, Marfan's syndrome, Bloom's syndrome.
• Exposure to toxic substances. Toxins are able to penetrate into the blood and cause the development of mutations. Chemical mutagens include antibacterial agents(chloramphenicol), cytostatics, benzene.
• ionizing radiation. Radiation radiation is partially absorbed by the cells of the human body, causing all sorts of disorders. At risk are residents of unfavorable (from an environmental point of view) areas and patients undergoing radiotherapy due to the presence of malignant neoplasms.

Pathology is a type of leukemia. The classification of the disease is based on the following factors:

• form of leakage: acute, chronic;
• type of development: true, relative (false);
• generation mechanism: primary, secondary.

Recently, on the basis of epidemiological observations, assumptions have been made about the relationship of the disease with the transformation of stem cells. There is a mutation of tyrosine kinase JAK 2 (Janus kinase), where in position 617 valine is replaced by phenylalanine. However, this mutation also occurs in other hematological diseases, but most often in 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 frequently detected mutation of the JAK2 tyrosine kinase gene with the replacement of valine by phenylalanine at position 617. Sometimes there is a family incidence of erythremia, for example, among Jews, which may indicate in favor of a genetic correlation.

With polycythemia in the bone marrow, there are 2 types of precursor cells of erythroid hematopoiesis: some of them behave autonomously, their proliferation is not regulated by erythropoietin; others, as expected, are erythropoietin-dependent. It is believed that an autonomous cell population 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 blood coagulation properties, and myeloid metaplasia of the spleen and liver. High blood viscosity causes a tendency to vascular thrombosis and hypoxic tissue damage, and hypervolemia causes increased blood filling of internal organs. At the end of polycythemia, depletion of hematopoiesis and myelofibrosis are noted.

General information

Polycythemia (Vakez's disease, erythremia, erythrocytosis) is a disease of the group of chronic leukemias, characterized by increased production of red blood cells, platelets and white blood cells, an increase in BCC, and splenomegaly. The disease is a rare form of leukemia: 4-5 new cases of polycythemia are diagnosed annually per 1 million population.

Erythremia develops mainly in patients of the older age group (50-60 years), somewhat more often in men. The relevance of polycythemia is due to the high risk of developing thrombotic and hemorrhagic complications, as well as the likelihood of transformation into acute myeloid leukemia, erythromyelosis, and chronic myeloid leukemia.

Clinical picture

The clinical manifestations of the disease are dominated by manifestations of plethora and complications associated with vascular thrombosis. The main manifestations of the disease are as follows:

• Expansion of skin veins and changes in skin color

On the skin of patients, especially in the neck area, protruding, dilated swollen veins are clearly visible. With polycythemia, the skin has a red-cherry color, especially pronounced on the exposed parts. body-to-face, neck, hands. The tongue and lips are bluish-red in color, the eyes are as if bloodshot (the conjunctiva of the eyes is hyperemic), the color of the soft palate is changed while maintaining the usual color of the hard palate (Kuperman's symptom).

Patients experience itching. Itching of the skin is observed in 40% of patients. This is a specific diagnostic feature for Wakez disease. This itching is aggravated after bathing in warm water, which is associated with the release of histamine, serotonin and prostaglandin.

These are short-term unbearable burning pains in the tips of the fingers and toes, accompanied by reddening of the skin and the appearance of purple cyanotic spots. The appearance of pain is explained by an increased number of platelets and the appearance of microthrombi in the capillaries. A good effect in erythromelalgia is observed from taking aspirin

A common symptom of erythremia is an enlargement of the spleen of varying degrees, but the liver may also be enlarged. This is due to excessive blood supply and the participation of the hepato-lienal system in the myeloproliferative process.

• Development of ulcers in the duodenum and stomach

Ulcer develops in 10-15% of cases duodenum, less often the stomach, this is associated with thrombosis of small vessels and trophic disorders in the mucous membrane and a decrease in its resistance to Helicobacter pylori.

• The occurrence of blood clots in the vessels

Previously, vascular thrombosis and embolism were the main causes of death in polycythemia. Patients with polycythemia have a tendency to form blood clots. This leads to impaired blood circulation in the veins. lower extremities, cerebral, coronary, splenic vessels. The tendency to thrombosis is explained by increased blood viscosity, thrombocytosis and changes in the vascular wall.

As well as increased clotting blood and thrombus formation in polycythemia, bleeding from the gums, from the dilated veins of the esophagus are observed

• Persistent joint pain and increased uric acid levels

Many patients (20%) complain of persistent arthritic pain in the joints, as there is an increase in the level of uric acid.

Many patients complain of persistent pain in the legs, the cause of which is obliterating endarteritis associated with erythremia and erythromelalgia.

• Pain in flat bones

When tapping flat bones and pressing on them, they are painful, which is often observed with bone marrow hyperplasia.

Deterioration of blood circulation in the organs leads to complaints of patients on fatigue, headache, dizziness, tinnitus, flushing of blood to the head, fatigue, shortness of breath, flies in the eyes, blurred vision. Arterial pressure increased, which is a compensatory response of the vascular bed to an increase in blood viscosity. Often develop heart failure, myocardiosclerosis.

Laboratory indicators for polycythemia vera

The number of red blood cells is increased and is usually 6 × 10¹²-8 × 10¹² in 1 l or more. Hemoglobin rises to 180-220 g / l, the color index is less than one (0.7-0.6). The total volume of circulating blood is significantly increased - 1.5 -2.5 times, mainly due to an increase in the number of red blood cells. Hematocrit (ratio of erythrocytes and plasma) changes dramatically due to an increase in erythrocytes and reaches a value of 65% or more.

The number of reticulocytes in the blood is increased to 15-20 ppm, which indicates enhanced regeneration of red blood cells.

Polychromasia of erythrocytes is noted, individual erythroblasts can be found in the smear.

The number of leukocytes increased by 1.5-2 times to 10.0 × 10 9-12.0 × 10 9 per liter of blood. In some patients, leukocytosis reaches higher numbers. The increase occurs due to neutrophils, the content of which reaches 70-85%. There is a stab, less often myelocytic shift. The number of eosinophils increases, less often basophils.

The number of platelets increased to 400.0×109-600.0×109 per liter of blood, and sometimes even more. Blood viscosity is significantly increased, ESR is slowed down (1-2 mm per hour).

Increases uric acid levels

Causes and classification

In hematology, there are 2 forms of polycythemia - true and relative. Relative polycythemia develops when normal level erythrocytes and a decrease in plasma volume. This condition is called stress or false polycythemia and is not considered within the scope of this article.

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

True polycythemia in its development goes through 3 stages: initial, advanced and terminal.

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

Stage II (erythremic, expanded) is divided into two substages:

• IA - without myeloid transformation of the spleen. There is erythrocytosis, thrombocytosis, sometimes pancytosis; according to the myelogram - hyperplasia of all hematopoietic sprouts, pronounced megakaryocytosis. The duration of the advanced 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.

Stage III (anemic, posterythremic, terminal). Characterized by anemia thrombocytopenia, leukopenia, myeloid transformation of the liver and spleen, secondary myelofibrosis. Possible outcomes of polycythemia in other hemoblastoses.

Treatment of complications of polycythemia

Complications of the disease occur due to thrombosis and embolism of arterial and venous vessels of the brain, spleen, liver, lower extremities, less often - other areas of the body. Development of splenic infarction ischemic stroke, heart attack, cirrhosis of the liver, deep vein thrombosis of the thigh. Along with thrombosis, bleeding, erosion and ulcers of the stomach and duodenum, anemia are noted. Very often, cholelithiasis and urolithiasis develop due to an increase in the concentration of uric acid. nephrosclerosis

For the prevention of thrombosis and embolism, disaggregation therapy is used: acetylsalicylic acid at a dose (from 50 to 100 mg per day), dipyridamole, ticlopedine hydrochloride, trental. At the same time, heparin or fraxiparin is prescribed. The use of leeches is ineffective. Applications have been found to reduce itching of the skin antihistamines- blockers of antihistamine systems H1 systems - (zyrtec) and paraxetine (paxil).

For iron deficiency, use:

• androgenic drugs: winebanin (Winobanin (Danazol®)
• erythropoietin
• thalidomide (alone or together with corticosteroid hormones)
• lenalidamide (revlemide) in combination with corticosteroid hormones

With the development of autoimmune hemolytic anemia, the use of corticosteroid hormones is indicated.

To reduce the level of uric acid - allopurinol, interferon α.

Bone marrow transplantation is rarely used for polycythemia because bone marrow transplantation itself can lead to poor outcomes.

With cytopenia, anemic and hemolytic crises, corticosteroid hormones (prednisolone), anabolic hormones, B vitamins are indicated.

Splenectomy is possible only in case of severe hypersplenism. If acute leukemia is suspected, surgery is contraindicated.

Symptoms of polycythemia

In the early stages of development, the disease does not detect itself in any way, so the person is not aware of its presence. As it progresses, the first symptoms of erythremia occur.

Initial

In the initial stages of the disease, there are no visible symptoms of Wakez Osler, so the patient is usually unaware of the presence of polycythemia in combination with the fact that the disease proceeds rather slowly. But when the disease intensifies, the first symptoms of Wakez's disease appear, namely, this is expressed in the appearance of migraine and dizziness, in the presence of a strange sensation inside the skull.

In the progressive stage of the disease, symptoms such as:

• Itching of the epidermis. Often, itching sensations increase after skin contact with water, which is caused by the release of histamine, prostaglandin and serotonin into the blood;
• Phlebeurysm, and a change in the shade of the epidermis. The epidermis in the area of ​​​​the physiognomy, hands and neck takes on a reddish tint. Also, upon careful study, you can notice a change in the color of the mucous membranes to dark red with blue. Due to varicose veins, swollen veins are clearly visible on the neck;
• Bleeding gums, do not forget that bleeding gums are a symptom of many more serious diseases;
• Enlargement of the liver and spleen;
• Colic in legs and joints.

There are several other symptoms of Wakez, which are less common, but can also indicate Wakez's disease: rapid asthenia, blurred vision, attention disorder, hearing damage, frequent mood changes, lethargy. Polycythemia is also associated with high pressure, uncontrolled weight loss, repeated colds.

Erythremia develops for a long time, gradually and can be detected by chance during a blood test. Early symptoms such as heaviness in the head, tinnitus, dizziness, blurred vision, chilliness of the extremities, sleep disorder etc., are often “written off” for advanced age or concomitant diseases.

Most feature polycythemia is the development of a plethoric syndrome caused by pancytosis and an increase in BCC. Evidence of fullness is telangiectasia, cherry-red coloration of the skin (especially the face, neck, hands and other open areas) and mucous membranes (lips, tongue), hyperemia of the sclera. A typical diagnostic sign is Cooperman's symptom - the color of the hard palate remains normal, and the soft palate acquires a stagnant cyanotic hue.

Another distinguishing symptom of polycythemia is pruritus, aggravated after water procedures and sometimes unbearable. Among the specific manifestations of polycythemia is also erythromelalgia - a painful burning sensation in the fingertips, which is accompanied by their hyperemia.

In the advanced stage of erythremia, excruciating migraines, bone pain, cardialgia, arterial hypertension. 80% of patients have moderate or severe splenomegaly; the liver increases a little less often. Many patients with polycythemia notice increased bleeding of the gums, bruising of 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 violation of the trophism of the skin and mucous membranes are trophic ulcers legs, stomach ulcers and duodenum. The most frequent complications in the clinic of polycythemia in the form of vascular deep vein thrombosis, mesenteric vessels, portal veins, cerebral and coronary arteries. Thrombotic complications (TELA, ischemic stroke, myocardial infarction) are the leading causes of death in patients with polycythemia. At the same time, along with thrombosis, patients with polycythemia are prone to hemorrhagic syndrome with the development of spontaneous bleeding of various localizations (gingival, nasal, from the veins of the esophagus, gastrointestinal and etc.).

Diagnosis algorithm

The algorithm for making a diagnosis is as follows:

1. Determine if the patient has:
   • A. increase in hemoglobin or B. increase in hematocrit
   • B. splenomegaly with or without an increase in platelets or leukocytes
   • D. portal vein thrombosis
2. If so, then secondary polycythemia should be ruled out.
3. If secondary polycythemia is excluded, continue the algorithm:

If the patient has three major criteria or the first two major ones in combination with two minor ones: then a hematologist consultation is necessary, since there are all data for the diagnosis of polycythemia vera.

Big Criteria:

• increase in the mass of circulating red blood cells:
  • for men - 36 ml / kg,
  • for women - more than 32 ml / kg
• saturation of arterial blood with oxygen (more than 92%)
• enlargement of the spleen - splenomegaly

Small (additional) criteria

• an increase in the number of platelets
• an increase in the number of leukocytes
• an increase in alkaline phosphatase
• increase in vitamin B 12

then there is a true polycythemia and observation by a hematologist is necessary.

Additionally, it is possible to determine the presence of growth of erythroid colonies in a medium without erythropoietin, the level of erythropoietin (analysis sensitivity 70%, specificity 90%), histology of bone marrow punctate,