Polycythemia disease. Other diseases from the group Diseases of the blood, blood-forming organs and certain disorders involving the immune mechanism

Hematologists know that this disease is difficult to treat and has dangerous complications. Polycythemia is characterized by changes in the composition of the blood that affect the health of the patient. How does the pathology develop, what are the symptoms? Learn diagnostic methods, treatment methods, medications, forecasts of life for the patient.

What is polycythemia

Men are more susceptible to the disease than women, middle-aged people are more likely to get sick. Polycythemia is an autosomal recessive disorder in which various reasons in the blood, the number of erythrocytes - blood cells increases. The disease has other names - erythrocytosis, polyhemorrhage, Wakez's disease, erythremia, its ICD-10 code is D45. The disease is characterized by:

splenomegaly - a significant increase in the size of the spleen;
increased blood viscosity;
significant production of leukocytes, platelets;
an increase in circulating blood volume (CBV).

Polycythemia belongs to the group of chronic leukemias and is considered a rare form of leukemia. True erythremia (polycythemia vera) is divided into types:

Primary - a malignant disease with a progressive form associated with hyperplasia of the cellular components of the bone marrow - myeloproliferation. Pathology affects the erythroblastic germ, which causes an increase in the number of red blood cells.
Secondary polycythemia is a compensatory reaction to hypoxia caused by smoking, alpine climbing, adrenal tumors, and pulmonary pathology.

Wakez's disease is dangerous with complications. Due to the high viscosity, blood circulation in the peripheral vessels is disturbed. IN large quantities uric acid is released. All this is fraught with:

bleeding;
thrombosis;
oxygen starvation of tissues;
hemorrhages;
hyperemia;
hemorrhage;
trophic ulcers;
renal colic;
ulcers in the digestive tract;
kidney stones;
splenomegaly;
gout;
myelofibrosis;
iron deficiency anemia;
myocardial infarction;
stroke
lethal outcome.

Types of disease

Wakez's disease, depending on developmental factors, is divided into types. Each has its own symptoms and treatment options. Physicians distinguish:

true polycythemia, which is caused by the appearance of a tumor substrate in the red bone marrow, leading to an increase in the production of red blood cells;
secondary erythremia - its cause is oxygen starvation, pathological processes that occur in the patient's body and cause a compensatory reaction.

Primary

The disease is characterized by tumor origin. Primary polycythemia is a myeloproliferative blood cancer that occurs when pluripotent stem cells in the bone marrow are damaged. When a disease occurs in the patient's body:

increases the activity of erythropoietin, which regulates the production of blood cells;
the number of erythrocytes, leukocytes, platelets increases;
there is a synthesis of mutated brain cells;
proliferation of infected tissues is formed;
there is a compensatory reaction to hypoxia - there is an additional increase in the number of erythrocytes.

With this type of pathology, it is difficult to influence mutated cells that have a high ability to divide. Thrombotic, hemorrhagic lesions appear. Wakez's disease has developmental features:

changes occur in the liver, spleen;
tissues are filled with viscous blood, prone to the formation of blood clots;
plethoric syndrome develops - cherry red color skin;
severe itching occurs;
increased blood pressure (BP);
hypoxia develops.

True polycythemia is dangerous for its malignant development calling severe complications. For this form of pathology, the following stages are characteristic:

Initial - lasts about five years, is asymptomatic, the size of the spleen is not changed. BCC increased slightly.
Extended stage - duration up to 20 years. Is different high content erythrocytes, platelets, leukocytes. It has two substages - without changes in the spleen and with the presence of myeloid metaplasia.

The last stage of the disease - posterythremic (anemic) - is characterized by complications:

secondary myelofibrosis;
leukopenia;
thrombocytopenia;
myeloid transformation of the liver, spleen;
cholelithiasis, urolithiasis;
transient ischemic attacks;
anemia - the result of depletion of the bone marrow;
pulmonary embolism;
myocardial infarction;
nephrosclerosis;
leukemia in acute chronic form;
hemorrhages in the brain.

Secondary polycythemia (relative)

This form of Wakez disease is provoked by external and internal factors. With the development of secondary polycythemia viscous blood, which has increased volumes, fills the vessels, provoking the formation of blood clots. At oxygen starvation tissue develops a process of compensation:

the kidneys begin to intensively produce the hormone erythropoietin;
active synthesis of erythrocytes in the bone marrow is launched.

Secondary polycythemia occurs in two forms. Each of them has features. The following varieties are distinguished:

stressful - caused in an unhealthy way life, prolonged overvoltage, nervous disorders, unfavorable working conditions;
false, in which the total number of erythrocytes, leukocytes, platelets in the analyzes is within the normal range, an increase in ESR causes a decrease in plasma volume.

Causes

The provoking factors for the development of the disease depend on the form of the disease. Primary polycythemia occurs as a result of a neoplasm of the red bone marrow. Predetermining causes of occurrence true erythrocytosis are:

genetic failures in the body - a mutation of the tyrosine kinase enzyme, when the amino acid valine is replaced by phenylalanine;
hereditary predisposition;
cancerous tumors bone marrow;
oxygen deficiency- hypoxia.

The secondary form of erythrocytosis is caused by external causes. play an important role in the development comorbidities. The provoking factors are:

climatic conditions;
living in highlands;
congestive heart failure;
cancerous tumors internal organs;
pulmonary hypertension;
action toxic substances;
overstrain of the body;
x-ray radiation;
insufficient supply of oxygen to the kidneys;
infections that cause intoxication of the body;
smoking;
bad ecology;
features of genetics - Europeans are more likely to get sick.

The secondary form of Wakez's disease is caused by congenital causes- autonomous production of erythropoietin, high affinity of hemoglobin for oxygen. There are also acquired factors for the development of the disease:

arterial hypoxemia;
kidney pathology - cystic lesions, tumors, hydronephrosis, stenosis renal arteries;
bronchial carcinoma;
adrenal tumors;
hemangioblastoma of the cerebellum;
hepatitis;
cirrhosis of the liver;
tuberculosis.

Symptoms of Wakez disease

The disease caused by an increase in the number of red blood cells and blood volume is distinguished by characteristic signs. They have their own characteristics depending on the stage of Wakez's disease. Common symptoms of pathology are observed:

dizziness;
visual impairment;
Cooperman's symptom - a bluish hue of the mucous membranes and skin;
angina attacks;
redness of the lower and lower fingers upper limbs accompanied by pain, burning;
thrombosis of various localization;
severe itching of the skin, aggravated by contact with water.

As the disease progresses, the patient develops pain syndromes various localizations. There are disorders of the nervous system. For the disease are characteristic:

weakness;
fatigue;
temperature increase;
enlargement of the spleen;
noise in ears;
dyspnea;
feeling of loss of consciousness;
plethoric syndrome - burgundy-red color of the skin;
headache;
vomit;
increase in blood pressure;
pain in the hands from touch;
chilliness of the limbs;
eye redness;
insomnia;
pain in the hypochondrium, bones;
pulmonary embolism.

initial stage

The disease is difficult to diagnose at the very beginning of development. Symptoms are mild, similar to a cold or a condition of the elderly, corresponding to old age. Pathology is detected by chance during tests. The symptoms of the initial stage of erythrocytosis are:

dizziness;
decrease in visual acuity;
headache attacks;
insomnia;
noise in ears;
sore fingers from touch;
cold extremities;
ischemic pain;
redness of mucous surfaces, skin.

Expanded (erythremic)

The development of the disease is characterized by the appearance of pronounced signs of high blood viscosity. Pancytosis is noted - an increase in the number of components in the analyzes - erythrocytes, leukocytes, platelets. The advanced stage is characterized by the presence of:

reddening of the skin to purple shades;
telangiectasia - pinpoint hemorrhages;
acute attacks pain;
itching, aggravated by contact with water.

At this stage of the disease, signs of iron deficiency are observed - stratification of the nails, dry skin. The typical symptom is strong increase the size of the liver, spleen. Patients have:

indigestion;
breathing disorder;
arterial hypertension;
pain in the joints;
hemorrhagic syndrome;
microthrombosis;
stomach ulcers, twelve duodenal ulcer;
bleeding;
cardialgia - pain in the left chest;
migraine.

With an advanced stage of erythrocytosis, patients complain of a lack of appetite. Investigations reveal stones in gallbladder. The disease is different

increased bleeding from small cuts;
violation of the rhythm, conduction of the heart;
puffiness;
signs of gout;
pain in the heart;
microcytoses;
symptoms urolithiasis;
changes in taste, smell;
bruising on the skin;
trophic ulcers;
renal colic.

anemic stage

At this stage of development, the disease passes into the terminal stage. body for normal functioning not enough hemoglobin. The patient has:

a significant increase in the liver;
progression of splenomegaly;
compaction of the tissues of the spleen;
at hardware research – cicatricial changes bone marrow;
vascular thrombosis of deep veins, coronary, cerebral arteries.

At the anemic stage, the development of leukemia is a danger to the patient's life. This stage of Wakez's disease is characterized by the occurrence of aplastic iron deficiency anemia, the cause of which is the displacement of hematopoietic cells from the bone marrow by connective tissue. In this case, symptoms are observed:

general weakness;
fainting;
feeling of lack of air.

At this stage, if left untreated, the patient quickly death. Thrombotic, hemorrhagic complications lead to it:

ischemic form of stroke;
thromboembolism pulmonary arteries;
myocardial infarction;
spontaneous bleeding - gastrointestinal, veins of the esophagus;
cardiosclerosis;
arterial hypertension;
heart failure.

Symptoms of the disease in newborns

If the fetus has suffered hypoxia during fetal development, its body, in response, begins to increase the production of red blood cells. A provoking factor in the appearance of erythrocytosis in infants is congenital heart disease, pulmonary pathologies. The disease leads to the following consequences:

the formation of bone marrow sclerosis;
impaired production of leukocytes responsible for immune system newborn;
development of infections leading to death.

At the initial stage, the disease is detected by the results of tests - the level of hemoglobin, hematocrit, red blood cells. With the progression of the pathology, already in the second week after birth, there are bright severe symptoms:

the baby cries from touch;
the skin turns red;
the size of the liver, spleen increases;
thrombosis appears;
body weight decreases;
analysis reveals increased amount erythrocytes, leukocytes, platelets.

Diagnosis of polycythemia

The patient's communication with the hematologist begins with a conversation, an external examination, and anamnesis. The doctor finds out heredity, features of the course of the disease, the presence of pain, frequent bleeding, signs of thrombosis. During the reception, the patient is diagnosed with polycythemic syndrome:

purple-red blush;
intense coloring of the mucous membranes of the mouth, nose;
cyanotic (cyanotic) color of the palate;
change in the shape of the fingers;
red eyes;
palpation is determined by an increase in the size of the spleen, liver.

The next stage of diagnosis is laboratory research. Indicators that indicate the development of the disease:

an increase in the total mass of erythrocytes in the blood;
increase in the number of platelets, leukocytes;
a significant level of alkaline phosphatase;
a large amount of vitamin B 12 in the blood serum;
increased erythropoietin in the secondary form of polycythemia;
decrease in situation (blood oxygen saturation) - less than 92%;
decrease in ESR;
increase in hemoglobin to 240 g / l.

For differential diagnosis pathology, special types of research and analysis are used. Urologist, cardiologist, gastroenterologist are consulted. The doctor prescribes:

biochemical analysis blood - determines the level uric acid, alkaline phosphatase;
radiological examination - reveals an increase in circulating red blood cells;
sternal puncture - fence for cytological analysis bone marrow from the sternum;
trepanobiopsy - histology of tissues from ilium, revealing three-growth hyperplasia;
molecular genetic analysis.

Laboratory research

The disease of polycythemia is confirmed by hematological changes in blood parameters. There are parameters that characterize the development of pathology. Data laboratory research indicating the presence of polycythemia:

Index	Units	Meaning
Hemoglobin
Mass of circulating red blood cells
Erythrocytosis	cells/ liter
Leukocytosis		over 12x109
thrombocytosis		over 400x109
Hematocrit

Serum vitamin B level 12
Alkaline phosphatase		over 100
Color indicator

Hardware diagnostics

After conducting laboratory tests, hematologists prescribe additional tests. To assess the risk of developing metabolic, thrombohemorrhagic disorders, hardware diagnostics are used. The patient undergoes research depending on the characteristics of the course of the disease. A patient with polycythemia is given:

Ultrasound of the spleen, kidneys;
heart examination - echocardiography.

Methods of hardware diagnostics help to assess the condition of the vessels, to identify the presence of bleeding, ulcers. Appointed:

fibrogastroduodenoscopy (FGDS) - an instrumental study of the mucous membranes of the stomach, duodenum;
ultrasound dopplerography (USDG) of the vessels of the neck, head, veins of the extremities;
CT scan internal organs.

Treatment of polycythemia

Before proceeding to therapeutic activities, it is necessary to find out the type of disease and its causes - the treatment regimen depends on this. The challenge for haematologists is to:

in primary polycythemia, prevent tumor activity by influencing the neoplasm in the bone marrow;
in the secondary form - to identify the disease that provoked the pathology and eliminate it.

Treatment of polycythemia includes drawing up a rehabilitation and prevention plan for a particular patient. Therapy includes:

bloodletting, which reduces the number of red blood cells to normal - 500 ml of blood is taken from the patient every two days;
maintaining physical activity;
erythocytophoresis - blood sampling from a vein, followed by filtration and return to the patient;
dieting;
transfusion of blood and its components;
chemotherapy to prevent leukemia.

In difficult situations that threaten the life of the patient, a bone marrow transplant is performed, a splenectomy is the removal of the spleen. In the treatment of polycythemia, much attention is paid to the use medications. The treatment regimen includes the use of:

corticosteroid hormones - severe course illness;
cytostatic agents- Hydroxyurea, Imiphos, which reduce the growth of malignant cells;
antiplatelet agents that thin the blood - Dipyridamole, Aspirin;
Interferon, which increases defensive forces enhancing the effectiveness of cytostatics.

Symptomatic treatment involves the use of drugs that reduce blood viscosity, prevent thrombosis, and the development of bleeding. Hematologists prescribe:

to exclude vascular thrombosis - Heparin;
with severe bleeding - Aminocaproic acid;
in the case of erythromelalgia - pain in the fingertips - non-steroidal anti-inflammatory drugs - Voltaren, Indomethacin;
with skin itching - antihistamines - Suprastin, Loratadin;
with an infectious genesis of the disease - antibiotics;
for hypoxic causes - oxygen therapy.

Bloodletting or erythrocytopheresis

An effective way to treat polycythemia is phlebotomy. When bloodletting is performed, the volume of circulating blood decreases, the number of red blood cells (hematocrit) decreases, and skin itching is eliminated. Features of the process:

before phlebotomy, the patient is administered Heparin or Reopoliglyukin to improve microcirculation, blood flow;
excess is removed with leeches or an incision is made, a puncture of a vein;
up to 500 ml of blood is removed at a time;
the procedure is carried out with an interval of 2 to 4 days;
hemoglobin is reduced to 150 g/l;
the hematocrit is adjusted to 45%.

Another method of treating polycythemia, erythrocytopheresis, is effective. With extracorporeal hemocorrection, excess red blood cells are removed from the patient's blood. This improves the processes of hematopoiesis, increases the consumption of iron by the bone marrow. Scheme for performing cytopheresis:

Create vicious circle- in a patient, the veins of both hands are connected through a special apparatus.
Blood is taken from one.P
It is passed through an apparatus with a centrifuge, a separator, filters, where some of the red blood cells are eliminated.
The purified plasma is returned to the patient - injected into a vein in the other arm.

Myelosuppressive therapy with cytostatics

In severe polycythemia, when bloodletting does not give positive results, doctors prescribe drugs that suppress the formation and reproduction of brain cells. Treatment with cytostatics requires ongoing blood tests to monitor the effectiveness of therapy. Indications are factors associated with polycythemic syndrome:

visceral, vascular complications;
skin itching;
splenomegaly;
thrombocytosis;
leukocytosis.

Hematologists prescribe drugs based on test results, clinical picture diseases. Contraindications for cytostatic therapy is children's age. Drugs used to treat polycythemia:

Myelobramol;
Imiphos;
Cyclophosphamide;
Alkeran;
Mielosan;
Hydroxyurea;
Cyclophosphamide;
Mitobronitol;
Busulfan.

Preparations for the normalization of the aggregate state of the blood

The objectives of treatment for polycythemia: normalization of hematopoiesis, which includes ensuring the liquid state of the blood, its coagulation during bleeding, restoration of the walls of blood vessels. Doctors have a serious choice of drugs so as not to harm the patient. Prescribe medications that help stop bleeding - hemostatics:

coagulants - Thrombin, Vikasol;
fibrinolysis inhibitors - Kontrykal, Amben;
vascular aggregation stimulants - calcium chloride;
drugs that reduce permeability - Rutin, Adroxon.

Of great importance in the treatment of polycythemia to restore the aggregate state of the blood is the use of antithrombotic agents:

anticoagulants - Heparin, Hirudin, Phenylin;
fibrolytics - Streptoliasis, Fibrinolysin;
antiplatelet agents: platelet - Aspirin ( Acetylsalicylic acid), Dipyridamole, Indobrufen; erythrocyte - Reogluman, Reopoliglyukin, Pentoxifylline.

recovery prognosis

What awaits a patient diagnosed with polycythemia? Forecasts depend on the type of disease, timely diagnosis and treatment, causes, and complications. Wakez's disease in its primary form has an unfavorable development scenario. Life expectancy is up to two years, which is associated with the complexity of therapy, high risks formation of strokes, heart attacks, thromboembolic consequences. Survival can be increased by using the following treatments:

local irradiation of the spleen with radioactive phosphorus;
lifelong bloodletting procedures;
chemotherapy.

A more favorable prognosis for the secondary form of polycythemia, although the disease may result in nephrosclerosis, myelofibrosis, erythrocyanosis. Although complete cure impossible, the patient's life is extended for a significant period - over fifteen years - provided:

constant monitoring by a hematologist;
cytostatic treatment;
regular hemocorrection;
undergoing chemotherapy;
elimination of factors provoking the development of the disease;
treatment of pathologies that caused the disease.

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Erythremia (polycythemia vera, erythrocytosis, Wakez disease)

What is Erythremia (polycythemia vera, erythrocytosis, Wakez disease) -

erythremia - chronic illness hematopoietic system of a person with a predominant violation of erythropoiesis, an increase in the content of hemoglobin and erythrocytes in the blood. Usually the disease appears at the age of 40 to 60 years, most often erythremia occurs in men. Children rarely get sick; family history of other cases of the disease is not noted.

What provokes / Causes of Erythremia (polycythemia vera, erythrocytosis, Wakez disease):

IN Lately on the basis of epidemiological observations, assumptions are made about the relationship of the disease with the transformation of stem cells. A mutation of tyrosine kinase JAK 2 (Janus kinase) is observed, where in position 617 valine is replaced by phenylalanine. However, this mutation also occurs in other hematological diseases, but with polycythemia most often.

Symptoms of Erythremia (true polycythemia, erythrocytosis, Wakez disease):

On early stages disease symptoms are usually absent. However, as changes in the blood supply system increase, patients complain of a vague feeling of fullness in the head, headaches, and dizziness. Other symptoms appear depending on which body systems are affected. Paradoxically, hemorrhage can be a complication of erythremia.

The expanded stage is characterized by a brighter clinical symptoms. The most frequent and hallmark are headaches, which sometimes have the character of excruciating migraines with visual impairment.

Many patients complain of pain in the region of the heart, sometimes in the form of angina pectoris, pain in the bones, in the epigastric region, weight loss, impaired vision and hearing, unstable mood, tearfulness. Common symptom erythremia is pruritus. Can be paroxysmal pain at the tips of the fingers and toes. Pain is accompanied by reddening of the skin.

On examination, a special red-cyanotic color of the skin with a predominance of a dark cherry tone attracts attention. There is also redness of the mucous membranes (conjunctiva, tongue, soft palate). Due to frequent thrombosis of the extremities, darkening of the skin of the legs is observed, sometimes trophic ulcers. Many patients complain of bleeding gums, bleeding after tooth extraction, bruising on the skin. In 80% of patients, there is an increase in the spleen: in the expanded stage it is moderately enlarged, in the terminal stage it is often observed severe splenomegaly. The liver is usually enlarged. Often in patients with erythremia, an increase in blood pressure. As a result of malnutrition of the mucous membrane and vascular thrombosis, ulcers of the duodenum and stomach may occur. An important place in the clinical picture of the disease is occupied by vascular thrombosis. Thrombosis of the cerebral and coronary arteries, as well as blood vessels, is usually observed. lower extremities. Along with thrombosis, patients with erythremia are prone to the development of hemorrhages.

In the terminal stage, the clinical picture is determined by the outcome of the disease - liver cirrhosis, coronary thrombosis, softening focus in the brain due to thrombosis cerebral vessels and hemorrhages, myelofibrosis, accompanied by anemia, chronic myeloid leukemia and acute leukemia.

The prognosis depends on the age of the patient at the time of diagnosis, treatment and complications. Mortality is high in the absence of treatment and in cases where erythremia is combined with leukemia and other cancers.

Diagnosis of Erythremia (true polycythemia, erythrocytosis, Wakez disease):

Of great importance in the diagnosis of polycythemia vera is the assessment of clinical, hematological and biochemical parameters illness. Characteristic appearance patient (specific skin color and mucous membranes). Enlargement of the spleen, liver, a tendency to thrombosis. Changes in blood parameters: hematocrit, the number of erythrocytes, leukocytes, platelets. An increase in the mass of circulating blood, an increase in its viscosity, low ESR, an increase in the content of alkaline phosphatase, leukocytes, serum vitamin B 12. It is necessary to exclude diseases where there is hypoxia and inadequate treatment with vitamin B 12.
To clarify the diagnosis, it is necessary to conduct trepanobiopsy and histological examination of the bone marrow.

The most commonly used indicators to confirm polycythemia vera are:

Increase in the mass of circulating red blood cells:
- for men - 36 ml / kg,
- for women - more than 32 ml / kg
Increase in hematocrit >52% in men and >47% in women, increase in hemoglobin >185 g/l in men and >165 g/l in women
Saturation arterial blood oxygen (over 92%)
Enlargement of the spleen - splenomegaly.
Weight loss
Weakness
sweating
No secondary erythrocytosis
Availability genetic abnormalities in bone marrow cells, except for the presence of a chromosomal translocation Philadelphia chromosomes or with rearranged BCR ABL gene
Colony formation by erythroid cells in vivo
Leukocytosis more than 12.0 × 10 9 l (in the absence of temperature reaction, infections and intoxications).
Thrombocytosis more than 400.0×10 9 per liter.
An increase in the content of alkaline phosphatase in neutrophilic granulocytes by more than 100 units. (in the absence of infections).
Serum vitamin B 12 level - more than 2200 ng
Low level erythropoietin
Bone marrow puncture and histological examination of punctate obtained by trephine biopsy show an increase in megakaryocytes.

Treatment of Erythremia (True polycythemia, erythrocytosis, Wakez disease):

Phlebotomy (bloodletting treatment) can rapidly reduce red blood cells. The frequency and volume of bloodletting depend on the severity of erythremia. After a series of such procedures, there is not enough iron in the body, which stabilizes the production of red blood cells and reduces the need for bloodletting.

In the advanced stage of erythremia, cytostatic therapy is indicated. The most effective cytostatic drug in the treatment of erythremia is imifos. The drug is administered intramuscularly or intravenously at a dose of 50 mg daily for the first 3 days, and then every other day. For the course of treatment - 400-600 mg. The effect of imifos is determined after 1.5-2 months, since the drug acts at the level of the bone marrow. In some cases, anemia develops, which usually gradually disappears on its own. With an overdose of imiphos, hematopoietic hypoplasia may occur, for the treatment of which prednisolone, nerobol, vitamin B6 and B12, as well as blood transfusions are used. Average duration remission is 2 years, maintenance therapy is not required. With a recurrence of the disease, sensitivity to imiphos persists. With increasing leukocytosis, rapid growth of the spleen, myelobromol is prescribed at 250 mg for 15-20 days. Less effective in the treatment of erythremia myelosan. As symptomatic remedies treatment of erythremia using anticoagulants, antihypertensive drugs, aspirin.

In severe cases, treatment methods that inhibit bone marrow activity are used. Previously, they used radioactive phosphorus or anti-cancer drugs, which sometimes led to the development of leukemia.

Prevention of Erythremia (true polycythemia, erythrocytosis, Wakez disease):

Which doctors should you contact if you have Erythremia (polycythemia vera, erythrocytosis, Wakez's disease):

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Other diseases from the group Diseases of the blood, hematopoietic organs and individual disorders involving the immune mechanism:

B12 deficiency anemia

Anemia due to impaired synthesis by utilization of porphyrins

Anemia due to a violation of the structure of globin chains

Anemia characterized by the carriage of pathologically unstable hemoglobins

Anemia Fanconi

Anemia associated with lead poisoning

aplastic anemia

Autoimmune hemolytic anemia

Autoimmune hemolytic anemia with incomplete heat agglutinins

Autoimmune hemolytic anemia with complete cold agglutinins

Autoimmune hemolytic anemia with warm hemolysins

Heavy chain diseases

Werlhof's disease

von Willebrand disease

Di Guglielmo's disease

Christmas disease

Marchiafava-Micheli disease

Rendu-Osler disease

Alpha heavy chain disease

gamma heavy chain disease

Shenlein-Henoch disease

Extramedullary lesions

Hairy cell leukemia

Hemoblastoses

Hemolytic uremic syndrome

Hemolytic anemia associated with vitamin E deficiency

Hemolytic anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-PDH)

Hemolytic disease of the fetus and newborn

Hemolytic anemia associated with mechanical damage to red blood cells

Hemorrhagic disease of the newborn

Histiocytosis malignant

Histological classification of Hodgkin's disease

DIC

Deficiency of K-vitamin-dependent factors

Factor I deficiency

Factor II deficiency

Factor V deficiency

Factor VII deficiency

Factor XI deficiency

Factor XII deficiency

Factor XIII deficiency

Iron-deficiency anemia

Patterns of tumor progression

Immune hemolytic anemias

Bedbug origin of hemoblastoses

Leukopenia and agranulocytosis

Lymphosarcomas

Lymphocytoma of the skin (Caesari disease)

Lymph node lymphocytoma

Lymphocytoma of the spleen

Radiation sickness

Marching hemoglobinuria

Mastocytosis (mast cell leukemia)

Megakaryoblastic leukemia

The mechanism of inhibition of normal hematopoiesis in hemoblastoses

Mechanical jaundice

Myeloid sarcoma (chloroma, granulocytic sarcoma)

multiple myeloma

Myelofibrosis

Violations of coagulation hemostasis

Hereditary a-fi-lipoproteinemia

hereditary coproporphyria

Hereditary megaloblastic anemia in Lesh-Nyan syndrome

Hereditary hemolytic anemia due to impaired activity of erythrocyte enzymes

Hereditary deficiency of lecithin-cholesterol acyltransferase activity

Hereditary factor X deficiency

hereditary microspherocytosis

hereditary pyropoykylocytosis

Hereditary stomatocytosis

Hereditary spherocytosis (Minkowski-Choffard disease)

hereditary elliptocytosis

Acute intermittent porphyria

Acute posthemorrhagic anemia

Acute lymphoblastic leukemia

Acute low percentage leukemia

Acute megakaryoblastic leukemia

The hematopoietic system in the body ensures its vital activity. All components of the human body, without exception, depend on the health of the hematopoietic system. Many have probably heard that there is a blood disease. There are many types of pathologies of the circulatory system and the causes of their occurrence. One of these is Wakez's disease, erythremia, or chronic leukemia - all these are identical terms related to malignant pathology of hematopoiesis. Now let's take a closer look at erythremia - what it is and the signs of this disease.

The pathogenesis of erythremia

Erythremia disease - what is it? Wakez's disease or erythremia, which, in fact, is related to the medical terms of one deviation in the activity of the circulatory system, is characterized by a critical increase in blood viscosity. Blood clotting occurs due to the growth shaped elements blood (mainly erythrocytes), a condition called polycythemia vera. It is also called primary polycythemia, a term derived from the Greek poly meaning many and citus meaning cell.

The etiology of erythremia - what is it? This pathological condition hematopoietic system, in which the bone marrow produces too many red blood cells. Due to the excess of red blood cells and some other blood cells, the blood becomes viscous. Thickened blood circulates through the vessels with difficulty, moreover, it is fraught with increased formation of blood clots. With such a development of events, about any full-fledged supply of organs and tissues with oxygen and nutrients out of the question. As a result of Vakeza's disease, hypoxia progresses, which only increases with time and entails whole complex health problems. Erythremia is divided into true and relative. True erythremia is accompanied by an increase in platelets and leukocytes.

What is erythremia was first discovered by Vakez in 1892, which is why the disease got his name. And only in 1903 it was finally established that due to malignant pathology an increase in the activity of the central organ of hematopoiesis, that is, the bone marrow.

It was the follower of Dr. Vakez, Osler, who singled out the disease into an individual nosological category of diseases of the hematopoietic system.

Symptoms of Wakez's disease in a predominant amount clinical cases occur in the elderly population, in particular males, aged 65 to 85 years. In elderly patients, erythremia occurs for a long time, often in latent form(chronic leukemia). Among the people in age period youth or maturity, Wakez disease is very rare. But those isolated cases that are detected indicate the rapid development of erythremia. And the acute form of Wakez disease occurs mainly in women.

Such an unusual etiology of erythremia, according to scientists in the field of medicine, may be due to a genetic predisposition. So, from a sick ancestor who had sluggish chronic leukemia, if the pathology was transmitted to descendants. In the younger generation, erythremia already proceeded in a faster form. But heredity is not the only cause of Wakez disease.

Causes of erythremia

Renal pathologies:

Hydronephrosis.
Stenosis of the renal arteries.
Kidney transplant.
cysts.

Erythropoietin-provoking tumors:

Hepatocellular carcinoma.
Oncology of the kidneys.
Ovarian cancer.
Pheochromocytoma.
Cysts and adenomas of the pituitary gland.
Tumor processes in the cerebellum.
Uterine myoma.

Stress erythrocytosis, also known as Gaisbeck's syndrome or pseudopolycythemia, refers to relative erythrocytes. With a relative form of erythremia, the erythrocyte sedimentation rate increases. Similar state due to a decrease in plasma volume, which also leads to an excess of the required erythrocyte mass. Clinical characteristics stress erythrocytosis covers obesity, high blood pressure, reactivity of the vascular system.

Stages of true erythremia

The first stage (initial) erythremia is characterized by the absence of symptoms. The person feels in the usual way, the hematopoietic system is still functioning normally, but the process of increased production of red blood cells is already beginning to gain momentum. At this stage of Wakez's disease, even if tests are taken, they will show an insignificant excess of all blood elements, except for leukocytes, which is why there may be an increased blood supply to all internal organs.

In this phase of erythremia, both the physician and the patient himself should not underestimate, at first glance, supposedly quite normal tests. Knowing about such a pathology of the hematopoietic apparatus as erythremia, it is better to play it safe and, in case of the slightest suspicion, continue research. This will help either dispel suspicions about the presence of Wakez disease, or start treatment in time to avoid severe consequences and significantly prolong the life of the patient. The initial stage of erythremia with inactivity can last about six years.

Starting from the second stage (A), the person already shows symptoms of erythremia. Wakez disease in this phase can be from 10 to 12 years. During this period of erythremia in a person, hematopoietic organs, such as the spleen and liver, gradually grow and increase in size. In blood tests for Wakez disease, iron deficiency is clearly visible.

IN vascular system with erythremia, thrombus formation can be observed.

Erythremia of the second stage (B) is characterized by a pronounced increase in the liver and spleen due to tumor processes. In the bone marrow with Wakez disease, you can see a lot of healed tissue. A patient who is in this period of progression of Wakez's disease may notice a tendency to bleed. And when examining the body, a large number of blood clots is easily detected.

The third stage of erythremia - anemic, occurs if the patient neglects the examination and treatment. The period of its development since the onset of erythremia is from 9 to 23 years. In addition to all the above deviations, gradually the blood cells cease to cope with the mission assigned to them, which entails many irreversible processes in the human body. One of these processes is large-scale scarring of brain tissue. At this stage of erythremia, the chances of staying alive are minimal. At constant surveillance doctors and continuous treatment of Wakez's disease, it is possible to maintain vital activity in the body for some time. But this state of affairs is unlikely to last long.

Clinical signs of erythremia

As it has already become clear from the previous section, that directly from the stage of erythremia, the symptoms are of varying degrees of severity. If the erythremia has not yet gained momentum, the person did not take blood tests and did not undergo examinations. In this case, Vaquez's disease can be suspected by the following signs.

Symptoms of erythremia (plethora):

Prolonged bleeding due to the slightest injury occurs due to the overflow of the body with blood and a violation of the clotting process.
Swollen veins appear (especially in the neck).
The skin acquires a cherry color (face, hands).
There is hyperemia of the lips and tongue (red-blue hue), as well as the conjunctiva of the eyeball.
Sensations of skin itching, which intensifies after water procedures.
Sometimes there may be a burning sensation at the ends of the fingers, nose, earlobes(erythromelalgia).
TO severe pain and burning are joined by cyanotic spots.

With erythremia, pain and burning in the limbs occur due to microthrombi, due to the formation of a large number of platelets. In Wakez disease, the symptoms may be in the form of duodenal ulcers. Duodenal ulcers accompany erythremia due to the formation of blood clots in the vessels of the gastrointestinal mucosa, which in turn lead to a deterioration in trophism. And the result of thrombus formation in the digestive organs is not only peptic ulcer, but also activation pathogenic bacteria Helicobacter pylori.

With the course of Wakez's disease, the patient progresses necrotic processes in the tissues of the upper and lower extremities. This pathological condition develops due to blockage of capillaries with a viscous blood substance. Necrotic changes in the tissues, they are first manifested by loss of sensitivity, become cold to the touch and pale. And when the process of saturation of tissues with nutrients and oxygen stops, these places turn black and are subject to excision or amputation.

As for the heart with Wakez disease, due to overflow of blood, there is a stretching (dilation) of the heart cavities. And as a result of thrombosis, cases of myocardial infarction or cerebral strokes, as well as pulmonary thromboembolism, are not uncommon. Therefore, at the first signs of erythremia, treatment should be started in order to avoid disability and save your life as long as possible.

Who is prone to erythremia

In the risk zone for the incidence of erythremia are persons:

With pathology of the respiratory system.
The presence of oncological processes.
subjected to constant stress.
With severe intoxication of the body.
If radiation exposure has taken place.
Due to major surgeries.
In case of violation of the structure of the hemoglobin protein (hemoglobinopathy).
With deviations in the endocrine system.
For heavy smokers.

Diagnosis of erythremia

Wakez disease can be diagnosed by donating blood for analysis and undergoing examination using instrumental devices.

Methods for diagnosing erythremia:

General clinical analysis of blood mass shows the level of erythrocytes, hemoglobin, platelets, leukocytes, hematocrit and ESR.
A biochemical study of the blood substance establishes indicators of uric acid, bilirubin, iron, alkaline phosphatase, AST (aspartate aminotransferase) and ALT (alanine aminotransferase).
A laboratory study of the blood flow of the TIBC reveals the level of total serum that binds iron.
The study of blood samples to establish the level of erythropoietin. By determining the indicators of this hormone, it is possible to detect the degree of deviations in the hematopoietic system.
Ultrasound (ultrasound) is used to examine the liver and spleen.

Bone marrow examination in Wakez disease by histological or cytological method. For this purpose, a trepanobiopsy or sternal puncture is performed. In addition to everything, it will be useful to conduct dopplerography. The diagnostic method shows the speed of blood flow in the studied organs and tissues. Thanks to Dopplerography in Wakez's disease, it is possible to identify areas of the circulatory system affected by blood clots.

Therapy for Wakez disease

Treatment of erythremia is reduced to maintaining the level of hematocrit and hemoglobin. Because it is not possible to cure Wakez's disease completely, due to hidden development. And at the time of the onset of symptoms, erythremia already has an irreversible process. But it is quite possible to stop and restrain the progression of both the true and the relative disease of Vaquez, naturally, under the vigilant control of the attending hematologist.

Erythremia treatment usually involves taking a number of drugs:

Iron-containing preparations to maintain hemoglobin levels.
Medicines to control the level of uric acid in the body.
Drugs that inhibit hyperplasia of germs in the bone marrow.
Drugs that reduce the formation of blood clots.
Preparations for the normalization of the spleen and liver.
Cytostatics, the action of which is aimed at suppressing tumor processes.

Drugs for the treatment of erythremia in the majority have serious side effects. It is strictly forbidden to take medicines for Wakez disease at your own discretion, since instead of therapeutic effect can cause irreparable harm to the body. The course of treatment and dosage of therapy for Wakez disease must be agreed with the attending physician. And only on the basis of the results of the survey, taking into account all individual characteristics organism.

In contact with

Wakez disease
This disease has many names: polycythemia, polycythemia vera, erythremia, and in honor of the authors who first discovered and described it in detail, Wakez disease or Wakez-Osler disease.
Osler linked the occurrence of polycythemia with an increase in bone marrow activity. The difference between this disease and erythrocytosis is the absence of damage to organs or systems of the human body, which are accompanied by a sharp increase in the number of red blood cells in the blood.
Polycythemia can be attributed to chronic leukemia, when the tumor affects all hematopoietic sprouts, to a greater extent affecting the erythrocyte. The disease is characterized by an increase in the number of not only erythrocytes, but also other blood cells(leukocytes, platelets), an increase in hemoglobin, viscosity and mass of circulating blood, as well as its coagulation. All this leads to disruption of the normal blood supply to organs and systems, hypoxia and the formation of blood clots.
The cause of the disease has not been established. However hereditary factor plays a role in its development. Disease long years can be asymptomatic, manifesting itself in old age (after 60-70 years), more often in males.
Clinical picture
The disease proceeds for a long time and has a relatively benign course. At the initial stage, the symptoms of the disease are mild, patients complain of headaches, dizziness, decreased performance, chilliness in the arms and legs, and sleep disturbance. In the future, itching appears, which is the main diagnostic sign of the disease and occurs in almost half of the patients. The color of the skin changes, it acquires a purple-cyanotic character, this is especially noticeable on the face, neck, hands. Thrombosis of the extremities not only causes discoloration of the skin, but also contributes to the formation of trophic ulcers. Often there are thrombosis of the vessels of the brain and heart, ulcers of the duodenum and stomach are formed. Almost all patients have splenomegaly (enlargement of the spleen). An increase in blood pressure with a simultaneous enlargement of the spleen is considered the hallmark of polycythemia.
The outcome of the disease can be thrombosis of various organs, anemia, acute leukemia, cirrhosis of the liver .
Diagnostics
The diagnosis of the disease is made on the basis of the clinical picture, laboratory data (hemogram, bone marrow histology). The number of red blood cells in 1 ml of blood increases to 7 - 10 million; hemoglobin reaches 180 - 240 g / l; hematocrit - more than 52% (in men) and more than 47% (in women).
Treatment
The tactics of treating patients is determined by the doctor depending on the manifestations of the disease and the stage of the disease.
The main methods include: bloodletting, the use of cytostatics, symptomatic therapy.

Polycythemia vera (erythremia, Wakez disease) refers to myeoproliferative diseases, during which, when a stem cell is damaged, the proliferation of its three sprouts occurs. going on advanced education erythrocytes, to a lesser extent - leukocytes and platelets. Almost always with this disease, myeloid metaplasia of the spleen is observed.

The number of cases of erythremia is 1 per 100 thousand of the population, and in men this pathology is somewhat more common than in women. Among people over 60 years of age, Wakez disease is more common than in other age groups.

Etiology and pathogenesis of polycythemia vera

The basis of the disease is the transformation of the hematopoietic stem cell, which gives the right to consider Wakez's disease as a clonal neoplastic disease.

A pluripotent stem cell undergoes malignant transformation, which leads to the involvement of all three hematopoietic lineages in the process. The cells of the erythroid germ reach the greatest proliferation. Since it occurs in the absence of erythropoietin, this feature is specific to erythremia.

A characteristic sign for Wakez's disease are also accumulations of polymorphic megakaryocytes.

With an increase in the mass of circulating red blood cells, the hematocrit increases, the blood becomes more viscous. The combination of these factors together with thrombocytosis leads to impaired microcirculation and frequent thromboembolic complications. In parallel, the development of myeloid metaplasia of the spleen occurs.

Clinical picture of Wakez disease

The disease proceeds in 4 stages reflecting the pathological processes occurring in the bone marrow and spleen.

In the first stage of the disease erythrocytosis is noted, in the bone marrow - panmyelosis. The duration of this steel is up to 5 years.

The disease in this stage is accompanied by plethora, acrocyanosis, burning pains and paresthesias in the fingertips. Some patients note the appearance of skin itching after washing. An increase in the volume of circulating erythrocytes leads to the appearance of arterial hypertension in patients who, before the onset of the disease, did not complain of this symptom, or aggravation of already existing hypertension, which is difficult to treat with traditional antihypertensive drugs. Symptoms of coronary heart disease, cerebral atherosclerosis become more pronounced.

Since the disease develops gradually, it takes from 2 to 4 years from its onset to diagnosis.

Stage II Wakez disease called erythremic and lasts from 10 to 15 years. In addition to erythrocytosis, there is an increase in the content of neutrophils with a shift to the left. The picture of the bone marrow at this stage of the disease is characterized by total three-growth hyperplasia with pronounced megakaryocytosis. Myeloid metaplasia of the spleen is still absent, but there is splenomegaly due to increased sequestration of erythrocytes and platelets.

This stage of the disease is accompanied by more severe and more frequent vascular complications. There is a higher risk of developing thrombotic complications in elderly patients with a history of previous thrombosis.

The most common thrombosed vessels of the brain, coronary arteries and vessels that feed the abdominal organs.

Often, with true polycythemia, despite an increased number of platelets, a hemorrhagic syndrome is observed, manifested by nosebleeds and profuse blood loss after tooth extraction. This is due to the slow conversion of fibrinogen to fibrin and impaired retraction of the blood clot.

Visceral complications in Wakez disease can be erosions and ulcers of the stomach and duodenum. They are the result of impaired blood flow and trophism of the mucous membrane of the stomach and intestines.

Second stage B erythremia characterized by the addition of myeloid metaplasia of the spleen. Splenomegaly becomes more pronounced. IN peripheral blood leukocytosis is observed, the shift of the formula to the left occurs to young forms. The bone marrow is characterized by panmyelosis. Some stabilization of the condition of patients at this stage of the disease may be due to a decrease in the level of erythrocytes due to their increased destruction by the spleen.

Third stage of Wakez disease called anemic. In addition to the phenomenon of anemia in the peripheral blood, thrombocytopenia and pancytopenia are observed. In parallel, splenomegaly and cachexia increase.

Diagnosis of erythremia

Criteria allowing diagnose polycythemia vera, are divided into main and additional.

The former include an increase in the mass of circulating red blood cells with normal saturation of arterial blood with oxygen, as well as splenomegaly.

Additional symptoms are thrombocytosis and leukocytosis in the absence of signs of infection, an increase in alkaline phosphatase activity above 100 units, high content vitamin B12.

Currently, great importance in the diagnosis of erythremia is attached to the picture of the bone marrow.

Treatment of true polycythemia

For the prevention of thrombosis, aspirin is prescribed at a dose of 50 to 250 mg per day.

Often, with the correction of microcirculation, the symptoms of skin itching disappear after hot bath. However, in some cases, to eliminate this symptom, one has to resort to ultraviolet irradiation autoblood.

To reduce the content of the number of red blood cells, bloodletting is used, which must be carried out very carefully. Frequent bleeding can lead to iron deficiency, which does not require correction without signs of a tissue deficiency of this element.

Severe erythremia requires the use medical methods decrease in the number of red blood cells. For this, hydroxyurea preparations, alpha-interferon, myelosan are used.

Despite the fact that the disease proceeds for a long time and relatively favorably, fatal outcomes in Wakez disease are possible due to developing complications diseases. The most common threat to the patient's life is thrombosis and thromboembolism, as well as the transformation of the disease into acute leukemia.

Prevention of true polycythemia

Preventive measures for erythremia are aimed at early detection diseases and prevent the development of complications of the disease.