Thrombotic thrombocytopenic purpura(TTP) is an autoimmune disorder associated with a severe deficiency of a von-Willebrand-factor-cleaving protease,termed ADAMTS-13,which cleaves the large von-Willebrand-multimers tha...
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Thrombotic thrombocytopenic purpura(TTP) is an autoimmune disorder associated with a severe deficiency of a von-Willebrand-factor-cleaving protease,termed ADAMTS-13,which cleaves the large von-Willebrand-multimers that are synthesized and secreted by endothelial *** ADAMTS-13 is not present,the resulting abnormally large von Willebrand factor multimers have a greater ability to react with platelets and cause the disseminated platelet thrombi characteristic of TTP.1 Neurologic symptoms,generally attributed to altered microvascular hemodynamics and concomitant metabolic derangements,are present in 60%of patients on initial examination and ultimately develop in about 90%of patients.2 We herein present an case of parainfectious TTP with multiple cortically-located infarctions which offers an opportunity to discuss and to shed light into the underlying mechanisms of neurological involvement in TTP.
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