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Zakrzepowa plamica maloplytkowa - patogeneza i leczenie

, : Zakrzepowa plamica maloplytkowa - patogeneza i leczenie. Acta Haematologica Polonica 37(Suppl. 4): 103-109

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. This syndrome is associated with congenital and acquired deficiency of ADAMTS13, a metalloprotease that cleaves ultra large von Willebrand factor multimers and reduces their adhesive activity. The acquired TTP is usually connected with production of autoantibodies against ADAMTS13. Plasma exchange is effective therapy for immunological TTP because it replenishes the deficient ADAMTS13 and removes pathogenic autoantybodies.

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