青年女性,分别先后因少年时不洁饮食及青年期妊娠7月发生急性肾功能不全、微血管病性溶血、血小板减少、癫痫反复发作.第一次肾活检为典型的急性血栓性微血管病变,经甲泼尼龙1.5g冲击、血浆置换2次后血小板升至正常,透析治疗半年后肾功能恢复；第二次发病重复肾活检见肾小球及血管呈慢性化病变,但存在急性肾小管-间质损伤,静脉滴注甲泼尼龙40 mg/d、输注血浆治疗3d后血小板即升至正常,1月后肾功能恢复.两次发病查血管性血友病因子裂解酶13(ADAMTS13)活性均正常、抗ADAMTS13抗体低滴度阳性.最终诊断为获得性血栓性血小板减少性紫癜.%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 histoiogiral showed classic acute thrombotic microangiopathies(TMA) .platelet increased to the normal after treatment of Prednisolone 0. 5 g/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.
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