裂体细胞性贫血、严重血小板减少症和肾功能不全：严重获得性ADAMTS-13缺乏所致血栓性微血管病。病例2。
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
作者信息
Winkler A, Kremer Hovinga J A, Bianchi V, Studt J-D, Lämmle B
机构信息
Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland.
出版信息
Hamostaseologie. 2003 Aug;23(3):103-8.
We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission. Pathomechanisms of thrombotic thrombocytopenic purpura, especially the role of ADAMTS-13, are discussed and therapeutic measures outlined.
我们报告了一例64岁女性因严重获得性ADAMTS-13（血管性血友病因子裂解蛋白酶）缺乏导致急性血栓性微血管病的病例。她通过血浆置换疗法和糖皮质激素成功治愈。七个月后复发，脾切除术后实现持久缓解。本文讨论了血栓性血小板减少性紫癜的发病机制，尤其是ADAMTS-13的作用，并概述了治疗措施。
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