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Polycythemia Vera

   Also found in: Dictionary/thesaurus, Medical, Acronyms, Wikipedia 0.01 sec.
polycythemia vera [‚päl·i‚sī′thē·mē·ə ′vir·ə]
(medicine)
An absolute increase in all blood cells derived from bone marrow, especially erythrocytes.

Polycythemia Vera 

(also erythremia, Vaquez’ disease), a chronic disease of the hematopoietic system first described by the French physician L. H. Vaquez in 1892. Polycythemia vera is characterized by an increase in erythrocyte, leukocyte, and platelet mass and in plasma volume. It is classified as a form of leukemia. The course of the disease is comparatively benign. The principal symptoms are a dark red coloration of the skin, elevated blood pressure, proneness to hemorrhaging and thrombosis, and enlargement of the spleen.

Cytological analysis of bone marrow is an important aid in diagnosis. Treatment includes phlebotomy and the administration of cytostatics.

REFERENCE

Vorob’ev, A. I., M. D. Brilliant. Patogenez i terapiia leikozov. Moscow, 1976.


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According to Izraeli, a similar mutation of the JAK2 in Down syndrome and leukemia causes Polycythemia Vera, a disease common in adults that leads to the overproduction of blood.
The main area of focus for the JAK2 program has been the development of orally available JAK2 inhibitors to treat myeloproliferative disorders, which include myelofibrosis, polycythemia vera (PV) and essential thrombocythemia (ET).
Although the JAK2 mutation linked to ALL differs slightly from the defect in polycythemia vera patients, research into JAK2 inhibitors already under way for that disease could speed drug development for ALL patients with Down syndrome, Izraeli says.
 
 
 
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