Thrombotic thrombocytopenic purpura; the case of successful treatment

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurological and renal abnormalities and fever. The pathogenesis TTP is related to a severe deficiency of specific metalloprotease, ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type I motif 13) that cleaves the largest von Willebrand factor multimers in plasma and prevents the spontaneous formation of platelet thrombi in the microcirculation. Prognosis of TTP has been improved by plasma therapy decreasing the mortality rate from 90% to less than 20%. A case of severe acute TTP in 39-year-old woman, successfully treated with repeated plasma exchange is presented. Recent data on pathogenesis and treatment of TTP are briefly reviewed.

тромботическая тромбоцитопеническая пурпура, гемолитико-уремический синдром, тромботическая микроангиопатия, фактор Виллебранда, ADAMTS 13, плазмообмен, иммуносупрессивные препараты, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, thrombotic microangiopathy, von Willebrand factor, ADAMTS 13, plasma exchange, immunosuppressive agents

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