Erythremia

Erythremia( true polycythemia) is a tumor of the hematopoietic tissue with a relatively benign course. Disturbance of hematopoiesis occurs at the level of the precursor cell of myelopoiesis. The main substratum of the tumor is mature red blood cells, but with the hyperplasia of all three

germs of hemopoiesis, the content of granulocytes and platelets can be increased.

Erythremia is characterized by total hyperplasia of the cellular elements of the bone marrow, especially the erythrocyte sprout. The ability of differentiation to mature forms is preserved. In the peripheral blood, pazzitosis is noted - an increase in red blood counts in combination with leukocytosis( neutrophilia) and thrombocytosis. The degree of increase in red blood counts depends on the duration of the disease. Increases the content of Hb( usually in the range 180-220 g / l) and erythrocytes( 6.8-8x1012 / L), which is accompanied by an increase in the Ht index, an increase in blood viscosity and a decrease in ESR, until the complete cessation of sedimentation, rheology,stasis. The number of leukocytes usually increases to 9-15x109 / l, in some cases( mainly in the presence of myeloid metaplasia of the spleen), leukocytosis can reach a fairly high level( 50x109 / L and higher).The leukocyte formula is often characterized by neutrophilia and rod-coteruclear shift, sometimes by a small eosinophilia. The number of platelets also increases, in some cases very significantly( over 1000x109 / l).Erythema with high thrombocytosis occurs more severely, more often accompanied by vascular complications. In the expanded stage of the disease, especially in the development of myeloid metaplasia of the spleen, qualitative changes in the cells of the red and white rows are recorded: polychromasia, anisocytosis, basophilic puncture of erythrocytes, normo-blastosis and toxic granularity of neutrophils. Puncture of the sternum with erythremia does not give a complete picture of the intensity of hematopoiesis, since punctate appears to be strongly diluted peripheral blood. The leycoerythroblastic ratio decreases due to a predominant increase in the erythrocyte germ, and the number of megakaryocytes increases. The trepanobiopsy method is more valuable, revealing the typical reduction of fatty tissue, hyperplasia of all three sprouts( panmyelosis), a significant increase in the size of megakaryocytes and increased platelet loosening. In the final stage of the disease, secondary myelofibrosis develops, or as a result of a tumor progression - blast crisis( more often as acute myeloblastic leukemia), hematosarcoma.