Primary polycythemia polycythemia rubra vera

This is a myeloproliferative syndrome of unknown cause leading to chronic proliferation of reticuloendothelial tissues in the liver, spleen, and bone marrow with excessive blood cell production. It most commonly affects middle-aged and elderly patients. The proliferative emphasis in hemopoiesis may involve red cells, white cells, and platelets, in differing balances, but the combination of abnormal red cell and platelet proliferation, particularly thrombocythemia, with platelet counts above 450 * 109/l is a very important cause of vascular occlusive complications in hospital patients. Typically, the patient has a ruddy complexion, generalized pruritus, particularly after taking a warm bath, hepatosplenomegaly, and digital and/or coronary and/or cerebral ischemia, and is at risk of intra-abdominal, intrcranial, coronary, and limb vascular occlusions. The blood findings show supranormal red cell, white cell, and platelet counts. The blood picture commonly shows evidence of iron-deficient erythropoiesis, with biochemical evidence of increased purine turnover in hyperuricemia. The bone marrow is hyperplastic with diagnostically useful megakaryocytic hyperplasia and pleomorphism, which are very conspicuous on histological examination of the bone marrow and marrow smears. Commonly, such patients present with vascular, neurological, or cardiological complications as a consequence of blood hyperviscosity and vascular occlusion affecting the digits, heart, and/or brain. If the importance of platelet counts above 450 * 10 9/l and/or red cell counts of 6 * 1012/l or more is overlooked, underlying myeloproliferation may be missed. Inappropriate surgical and/or medical treatment, which fails to deal with the hematological disease upon which the clinical syndromes are based, may then be given. These patients are, of course, a minority of those presenting with vascular insufficiency and ischemia, but, because of the remediable nature of the myeloproliferation, it is particularly important to make the diagnosis, preferably at first presentation. Such patients may have no further vascular occlusive or ischemic organ complications once the myeloproliferation is treated. Hematological examinations upon which diagnosis depend include blood count, measured blood volume indices, and marrow examination—the last of these is particularly important when the red cell volume is raised.

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