关键词: 微血管病性溶血  , 血小板减少  , 急性肾损伤  , 血栓性血小板减少性紫癜  , 血管性血友病因子裂解酶13

Abstract:

 ABSTRACT  A female patient was admitted for both presenting as acute renal dysfunction, microangiopathic hemolysis, thrombocytopenia, epilepsia and transient fever separately when she was 14 and 22 years old. In her first onset, renal histological showed classic acute thrombotic microangiopathies（TMA）, platelet increased to the normal after treatment of Prednisolone 0.5g/d for 3 days and plasma exchange twice, her renal function recovered after half-year hemodialysis. Her second renal biopsy showed that glomcrulus and blood vessel displayed mainly chronic pathological changes, meanwhile obviously acute tubulointerstial injury. Platelet increased and renal function recovered quickly. ADAMTS13 activities were normal, and anti-ADAMTS13 antibodies were positive at her two episodes. Finally, TTP was diagnosed.

Key words: microangiopathic hemolysis , thrombocytopenia , acute kidney injury , TTP , ADAMTS13