皮肤淤斑、血小板减少伴肾功能损害  

Purpura,thrombocytopenia,anemia and acute renal failure

作　　者：涂远茂 谢红浪[1,2] 刘志红 

机构地区：[1]全军肾脏病研究所学术委员会 [2]南京军区南京总医院全军肾脏病研究所,南京210002

出　　处：《肾脏病与透析肾移植杂志》2011年第3期290-295,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：47岁男性患者,急性起病,临床表现为微血管病性溶血性贫血、血小板减少、高血压及肾功能损害,补体C3减低,血浆ADAMTS13活性极度缺乏,肾活检证实病理改变为血栓性微血管病;大剂量血浆及甲泼尼龙(累计5g)治疗后血液学缓解,ADAMTS13酶活性恢复正常,但病情反复,肾功能继续恶化,8个月后进入维持性血液透析。A 47-year-old man with abrupt onset trilogy manifesting thrombocytopenia and microangiopathic hemolytic anemia and acute renal failure, accompanying with massive proteinuria, microscopic hematuria, low serum complement C3 and extreme deficiency of ADAMTS13 activity, was proved as thrombotic microangiopathy （TMA） by renal biopsy pathology. Although large amount plasma exchange and methylprednisolone pulse therapy was administrated, the hemolysis anemia was remission, but the TTP relapsed repeatedly and the renal function deteriorated progressively. Eight months later, he developed to ESRD, and was treated with maintenance hemodialysis.

关 键 词：血栓性微血管病 血栓性血小板减少性紫癜 ADAMTS 13 非典型溶血尿毒综合征 

分 类 号：R554.6[医药卫生—血液循环系统疾病]