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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。
Young women, respectively, because of juvenile unclean diet and adolescent pregnancy occurred in July acute renal insufficiency, microangiopathy hemolysis, thrombocytopenia, recurrent epilepsy. The first renal biopsy is a typical acute thrombotic microvascular disease, the impact of methylprednisolone 1.5g, plasma exchange 2 times after platelets rose to normal, six months after dialysis treatment of renal function recovery; the second incidence of repeated renal biopsy see the kidney Balls and blood vessels were chronic disease, but there is acute tubular-interstitial injury, intravenous methylprednisolone 40 mg / d, plasma platelet 3 days after infusion of plasma rose to normal after 1 month of renal function recovery. The two pathogenesis of von Willebrand factor lyase 13 (ADAMTS13) activity were normal, low anti-ADAMTS13 antibody titer positive. The final diagnosis of acquired thrombocytopenic purpura.