Polycythemia

Polycythemia (synonym disease Wekesa) is a chronic disease of the blood, characterized by a persistent increase in the number of red blood cells, the total volume of blood, the enlargement of the spleen and increased production in the bone marrow, not only of red blood cells, but also of leukocytes and platelets.
The polycythemia refers to the group of leukemia. Autopsy revealed a sharp plethora of internal organs, often vascular blood clots, heart attacks, hemorrhage. In the bone marrow phenomena hyperplasia (increase cellular elements) eritropoeticescoe Rostock, diatezah bones - turning fat bone marrow in red.
Polycythemia develops gradually and has a progressive course. Clinically expressed deep red colour with a cyanotic shade, a plethora of mucous membranes with possible bleeding from the gums, stomach, intestines, uterus, spleen, and liver, hypertension. In the blood increased content of erythrocytes (6 000 000 - 10 000 000), hemoglobin (20-23 g%), ROE slowed down up to 1 mm per 1 or even 2 hours.
During the process of long-term, the Outlook is deteriorating, if develop thrombosis blood to vital organs.
Treatment in the hospital again bloodletting, radioactive phosphorus, cytotoxic drugs (mielosan, imifos, mailobama).

Polycythemia rubra, true (polycythaemia, rubra, vera; from the Greek. poly - lot, kytos - cell and haima, blood; synonym: eritremia, disease Wekesa) is a chronic disease of the blood device of unknown etiology characterized by a persistent increase in the number of red blood cells and total blood volume with the expansion of blood, spleen, and increased activity of the bone marrow, and hyperplastic process applies not only erythropoiesis, but also leiko - and thrombocytopoiesis.
Recently established neoplastic theory of pathogenesis. The polycythemia is considered a separate disease and referred to the group of myeloproliferative leukemia, considering as chronic erythromelas (see) with primary function strengthening of erythropoiesis.
Autopsy revealed a sharp plethora of internal organs, often vascular blood clots, heart attacks, gemorragii. The spleen is enlarged, hard, dark blue-red color. The liver is often enlarged, can be zirroticeskih changed. In diatezah bones - turning fat bone marrow in red. Hyperplasia eritropoeticescoe Rostock in the bone marrow and extramedullary foci blood saves the usual type of regeneration, hyperplasia of myeloid tissue sometimes becomes similar to leukemia. Significant hyperplasia megakaryocytes apparatus. These changes are identified and in the clinic when sternal puncture and more clearly if trepanobiopsy of the Ilium.
Clinical course and symptoms. Polycythemia develops most often in the elderly (40-60 years), however, documented cases of the disease in the young, and even in childhood. The disease usually develops gradually. The life expectancy of patients from the moment of detection of the disease now reaches an average of 13.3 years [Lawrence J. N. Lawrence)], and in some cases even up to 30 years or more (that is, D. Oak and M. A. Yasinovka).
Typical special colouring of the coat (eritro): intense dark red with cherry tone color of the skin, especially on the face and distal parts of the limbs; the mucous membranes of bright red color, often cyanotic; noticeable vascular injection sclera, gums loosened, often bleed discovered phenomenon of periodontal disease. The plethora with the increase of the weight of the circulating blood is 2-4 times, with high viscosity significantly affects the condition of the cardiovascular system and blood circulation, blood flow velocity decreases by 2-3 times and more. Hypertension is one of the most important and frequent symptoms of polycythemia. We should not exclude the combination of polycythemia with hypertension. Important lesions of peripheral vessels with the development of obliterating thrombangiitis, and sometimes clogged arteries with gangrene, thrombosis of cerebral vessels, coronary arteries, splenic and renal arteries with education infarction, thrombosis of the portal vein and its branches. There are bleeding from the nose, gums, stomach, intestines, uterus, and others, bleeding in the brain, stomach, spleen.
Disorders of the nervous system arise from the very beginning of the disease. On set of neurological symptoms you can highlight a specific syndromes: insufficiency of cerebral blood circulation, neurasthenic, diencephalic, vegetative-vascular, polyneuritises and erythromelalgia.
Splenomegaly is observed in 2/3-3/4 of all cases. Increase and compaction of the liver is noted in 1/3-1/2 of patients.
Pronounced changes in the condition of the kidneys is not observed.
The number of erythrocytes in 1 ml blood is usually 6-10 million, in some cases 12 million Percentage of reticulocytes is relatively low. Hemoglobin reaches 120-140% (20-23 g%), rarely above. Color indicator is lower units. The white blood cell count elevated (more than 1/2 of patients), and sometimes reaches 20 000-25 000 and more than 1 mm3 mainly due to Klebsiella with a shift to the left until metamyelocytes and plasmic order has been revealed. The largest number of leukocytes and the emergence of newer forms of the notes in the development myeloid leukemia. Increases for the most part and the number of platelets - up to 600 000 and even sometimes up to 1 million or more in 1 mm3. ROHE slowed down up to 1 mm per 1 or even 2 hours. The ratio between the volume weight of erythrocytes and plasma determined by the hematocrit increases to 85:15. Frequent pain in the bones were changes in their tissues, especially in epimutation long tubular bones.
In the early stages of diagnostic importance appearance of the neurovascular disorders. When expressed picture polycythemia recognition is primarily based on the classic triad of retros, oligopoly, splenomegaly. One must distinguish polycythemia from a number of States, is also characterized by the increase in the number of red blood cells per volume of blood - the so-called oligopoly, or erythrocytosis. False polyglobular not related to the actual increase in the number of red blood cells in the peripheral blood, and occurs due to thickening of the blood, for example if significant diarrhea and vomiting (e.g. cholera), increased sweating, excessive diuresis. Symptomatic polyglobular can be relative, when increasing the number of red blood cells in the peripheral blood mainly at the expense of redistribution (vicodinii deposited blood), for example, with the rapid rises to the height of acute cardiac and pulmonary insufficiency.
Of particular importance in differential diagnosis becomes true absolute polyglobular with reactive increase bone marrow erythropoiesis. Most often it is associated with long anoxic condition: the residents of the Alpine areas, with congenital heart defects, acquired valvular diseases with severe circulatory insufficiency, sclerosis of the branches of a pulmonary artery, pneumosclerosis, pronounced emphysema, and other lung diseases. This includes poligonului when exposed to toxic substances in the blood. Gaining importance in the occurrence of oligopoly and Central nervous system (for example, subthalamic region) inflammation or tumor process, some endocrine disorders (the syndrome Itsenko-Kushinga) and others In differential diagnosis between polycythemia and oligopolies in favor of polycythemia show enlargement of the spleen, leukocytosis with neutrophilic shift to the left, thrombocytosis, a significant increase in the total mass of blood and especially red blood cells with high hematocrit, the data of trepanobiopsy, a significant increase of alkaline phosphatase activity of neutrophils, a high rate of absorption of plasma and other Fe69
The forecast, given the progressive nature of the disease, the lack of spontaneous remissions and spontaneous cure, generally unfavourable, although modern treatment of life and health persist longer. The cause of death are often vascular complications - thrombosis, bleeding, bleeding, circulatory failure or a transition to a picture mieloza or, more rarely, genocidalists, aplastic anemia due to the development of myelofibrosis and osteomalacia.


The pathogenetic treatment. Bloodletting (usually 400-500 ml repeated at intervals 2-3-5 days to clear decrease in red blood) are especially recommended for high blood pressure, the threat of cerebral complications and high hematocrit. This method provides relief only in the coming months, often used in combination with radiofosfornuyu therapy.
The most effective radiation therapy. More appropriate radiation-ray view of the whole body.
In recent years, is widely used radioactive phosphorous (P32), which is administered on an empty stomach through the mouth in the form NaHP32O4 in 20-40 ml of 40% glucose solution, you can apply and intravenously. Contraindications to the use P32 - liver disease with significant dysfunction, diseases of kidneys, lakopenia (below 4000 1 mm3), thrombocytopenia (less than 150 000 1 mm3).
More widespread fractional introduction P32 (on reception 1.5 - 2 mkori once in 4-7-10 days, at the rate of 6-8 mkori in accordance with the indicators of red blood and weight of the patient). Before treatment P32 recommend to spend 2-3 bloodletting 400-500 ml at intervals of 2-3 days, especially in patients with pronounced symptoms of cerebral circulation, quantity of erythrocytes above 7.5-8 million in 1 mm3 and higher hematocrit (65 - 70).
Clinical effect effect after 2-4 weeks, and hematological remission occurs within 2-4 months. after the start of treatment and usually lasts 2-3 years or more.
When treating P32 may experience complications such as leukopenia, thrombocytopenia and anemia less with the transient nature.
Repeated courses of treatment P32 appoint relapses of the disease.