Polycythemia

Polycythemia, first described at the beginning of the XIX century, is characterized by an increase in the number of red blood cells, hemoglobin and total red blood cell (OOE). There are two main forms of polycythemia - a true and relative. Red cell mass increases only in polycythemia vera. This phenomenon has been observed in two situations: when a primary polycythemia and secondary polycythemia. The latter usually occurs as a result of increased activity of erythropoietin, which may result as an appropriate physiological response to reduced blood oxygen saturation, and inadequate production of hormones. When the relative polycythemia red cell mass does not exceed normal levels, and increase OOE is a consequence of the anomalous decrease of the plasma volume or increasing the number of red blood cells and reduce the volume of plasma in the normal range of these parameters.

The classification of polycythemia

Polycythemia vera

primary

secondary
# Chronic lung disease
# Kidney disease - for example, tumors, cysts (single or multiple), hydronephrosis
# Tumors - for example, cerebellar hemangioma, fibroma of the uterus
# Other reasons - for example, lifting height, the abnormal hemoglobin, smoking

Relative polycythemia (also called false stressful psevdotsitemiey, Gaysbeka syndrome)

Polycythemia vera

Polycythemia - a chronic progressive myeloproliferative disease characterized by an absolute increase in red cell mass, 2 / 3 of patients have an increase in the number of leukocytes and platelets.

Etiology

The disease results from mutations polypotent stem cells. Studies of bone marrow in vitro, as well as exploring options for glucose-6-phosphate dehydrogenase in women with polycythemia vera, indicate the presence of the disease in two populations of erythroid progenitor cells. Cells of one of the populations of autonomous and proliferate even in the absence of erythropoietin, whereas another population behaves normally, remaining eritropoetinzavisimoy. Thus, the first population most likely represents the autonomous mutant clone [Adamson, 1970; Golde, 1977].

Levels of erythropoietin in urine and plasma of patients with polycythemia vera range brand name viagra from normal values to zero and increases as appropriate, after bloodletting. Low levels of erythropoietin are due to inhibition of its production according to the type of feedback due to increased red cell mass.

Prevalence

Polycythemia - a rare disease. U.S. researchers have shown that each year 4-5 new cases per 1 million population [Modan, 1965]. The disease affects people of middle and old age, peak incidence occurs in the age group 50-60 years. The average age of first cases gradually increased from 44 years in 1912 to 60 years in 1964, men suffer more often than women (1.5: 1). The influence of racial factors is controversial. Modan (1965) found that among the Jews of incidence higher than expected, but lower than among blacks, but these observations were not confirmed.

Clinical manifestations

The symptoms of this disease is partly due to excessive production of red blood cells and platelets, which leads to an increase in blood viscosity and vascular thrombosis, and partly due to an increase in blood volume, which increases the blood flow of various organs.

The disease develops gradually, and so slowly that the diagnosis can be made accidentally when examining a patient about an entirely different disease. Symptoms are often nonspecific, such as headache, dizziness, tinnitus, confusion, irritability, blurred vision, they can easily be explained by old age or other, more common in older diseases. However, polycythemia can be suspected by the appearance of the patient - the red color of the skin and mucous membranes with cyanotic tinge in cold weather, telangiectases on his face, scleral hyperemia, and retina. Spleen in most cases, moderately enlarged, the liver is usually increased slightly. Sometimes there pain in the sternum.

The main symptoms are manifested in the event of thrombosis or, what happens less often, bleeding. Most often affects the blood vessels of the central nervous system that leads to a transient ischemic disorders, cerebrovascular accidents and visual impairment in the form of scotoma, diplopia, and temporary blindness. As a result of the defeat of the cardiovascular system may experience shortness of breath on exertion, angina and myocardial infarction. Peripheral vascular disease manifested rodonalgia, arterial and venous thrombosis, Raynaud's phenomenon, and even gangrene. When circulatory disorders of the gastrointestinal tract, changes occur indigestion, flatulence, and 1 in 10 patients - peptic ulcer. Thrombosis of the portal vein leading to portal hypertension and bleeding from esophageal varices.

About 1 in 10 patients, usually men, found clinical signs of gout, whereas hyperuricemia observed in 1 / 3 of all patients and is due to enhanced synthesis and breakdown of nucleoproteins. The frequency of these complications increases with disease duration. In the kidney stones may form, and in the joints - gouty deposits. In the family history of gout patients are rare.

About 2 / 3 of patients suffering generalized itching, which occurs, apparently as a result of histamine release from basophilic granulocytes. Especially painful itching hands and feet, it is amplified after washing and a warm bed.

Laboratory data

The number of red blood cells increased to 6,5-7,5 x 1012 per liter. Hemoglobin level increased to 180-240 g / liter. OOE usually above 0.52. Because based on measurements of hemoglobin, OOE and the number of red blood cells to accurately calculate the mass of red Viagra and penile erection blood cells is not possible, it should be measured by direct method using the radionuclide label. Red cell mass in excess of 36 ml / kg in males and 32 ml / kg in females indicates the presence of polycythemia.

In a smear of peripheral blood red cells have normal morphology, but because of frequent iron deficiency caused by repeated bleeding or bleeding, can be observed microcytosis. Leukocyte count increased to 12-30 ? 109 / L in about 2 / 3 patients, a higher leukocytosis occurs in later stages of the disease.

Sometimes there is a slight shift of the leukocyte formula to the left, rarely detected in peripheral blood of metamyelocytes, myelocytes, or even cells of earlier stages.

Alkaline phosphatase activity of leukocytes (SCHFL) increased. In 70-90% of patients with active SCHFL reaches 100-350, but in some cases, especially in the early stages of the disease, it remains within normal limits.

Platelet count at the time of diagnosis increased from 65% of patients, reaching 400-800 x 109 per liter. Very rarely observed and much higher values. Sometimes found morphological and functional abnormalities. Platelets can be increased in size and have a bizarre shape, and sometimes there are fragments of megakaryocytes. Functional disorders of platelets, are probably the main cause of hemorrhagic complications occurring in this disease. The content of vitamin Bj2 in serum and its ability to bind the vitamin is often elevated.

Hyperplastic bone marrow. Erythropoiesis normoblastny. Edge of normoblasts may be serrated, and vacuolated cytoplasm, which is especially common in patients treated with phlebotomy, and indicates iron deficiency. Revealed by staining free iron may be completely absent.

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