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Polycythaemia vera (PV) is generally a disease of middle and older age. Carries increased risks of thrombosis, haemorrhage, progression to myelofibrosis, and transformation to acute leukaemia. Survival is shorter than that of the general population; leading cause of death is cardiovascular complications (including thrombosis and haemorrhage).
Polycythemia vera is a condition characterized by an increased number of red blood cells in the bloodstream (erythrocytosis). Affected people may also have excess white blood cells and platelets. Conditions where the body makes too many of these cells are known as myeloproliferative neoplasms. These extra cells cause the blood to be thicker
JAK2-negative polycythaemia vera (requires A1-A4 plus another A or two B criteria) a a This is a very rare clinical entity. A1 Raised red cell mass (>25% above predicted) OR haematocrit ≥0·60 in men, ≥0·56 in women: A2 Absence of mutation in JAK 2: A3 No cause of secondary erythrocytosis: A4 Bone marrow histology consistent with
Polycythaemia. Polycythaemia can be relative (decreased plasma volume) or true polycythaemia. True polycythaemia can be primary (the myeloproliferative neoplasm Polycythaemia vera) or secondary (to states associated with appropriate or abnormal excessive erythropoietin. Review clinical findings, including history of smoking and diuretic therapy.
There is no cure, but treatment can extend the person’s life span by thinning the blood and reducing the risk of blood clots and other complications. Options include: Weekly removal of one unit of blood until red blood cells make up less than 50 per cent of the overall blood volume. This procedure is called either a phlebotomy or venesection.
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