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Since the original description of a syndrome of pallor, purpura, and stroke by Moschcowitz in 1924, this constellation of clinical characteristics has been observed in a number of disorders, having thrombotic occlusion of small vessels in common. These diverse syndromes have now been defined and classified, and their diagnostic features and treatment described in a review article by George and Nester.
Thrombotic thrombocytopenic purpura (TTP) is due to genetic mutation or autoantibody against ADAMTS13, a von Willebrand factor–cleaving protease. The persistence of large, uncleaved von Willebrand factor multimers is associated with occlusion of small vessels by platelet thrombi. The salient laboratory finding is a level of ADAMTS13 that is <10…