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T HROMBOTIC T HROMBOCYTOPENIC P URPURA AND H EMOLYTIC U REMIC S YNDROME (TTP-HUS) Introduction: TTP-HUS is a multi-system disorder characterized by thrombocytopenia (TCP), microangiopathic hemolytic anemia (MAHA) and ischemic manifestations due to platelet aggregation in arterial microcirculation. There has been a distinction between the 2 disorders in the past because of their different clinical presentations (TTP with more neurological manifestations and HUS with more renal failure); however, they are thought now to have the same underlying pathology. Although rare, this is an important disorder due to its abrupt onset and high mortality rate if untreated. Plasma Exchange has decreased the mortality from >90% to 10-20%. I. Incidence: uncommon, estimated at 3.7/million and on the rise. II. Pathogenesis: Remains unclear, however, the leading hypotheses state that two events can initiate the disorder: abnormal platelet aggregation OR endothelial cell injury. Both will cause platelet microthrombi in arterioles and capillaries (sparing venules) leading to a consumptive thrombocytopenia. Intravascular hemolysis ensues as red blood cells are fragmented as they pass through narrowed microvascular lumen. a.
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This note was uploaded on 11/23/2011 for the course DIAG 2735 taught by Professor Josephr.forese during the Winter '11 term at Life Chiropractic College West.

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