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一名患有自身免疫性肝炎和系统性红斑狼疮的女性获得性甲型血友病:文献综述

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus: review of literature.

作者信息

Rezaieyazdi Zahra, Sharifi-Doloui Davood, Hashemzadeh Kamila, Shirdel Abbas, Mansouritorghabeh Hassan

机构信息

Rheumatic Disease Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

出版信息

Blood Coagul Fibrinolysis. 2011 Dec;22(8):738-41. doi: 10.1097/MBC.0b013e32834a5c8e.

Abstract

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.

摘要

继发于系统性红斑狼疮(SLE)的获得性甲型血友病是一种罕见的出血素质。在此,我们报告一名37岁患有自身免疫性肝炎的女性,她患上了SLE,并出现由VIII因子(fVIII)抑制剂导致的获得性血友病。她表现为自发性瘀斑和血尿。活化部分凝血活酶时间延长,fVIII活性降低,且fVIII抑制剂滴度高。治疗方案开始采用静脉注射甲泼尼龙冲击治疗,随后继续使用泼尼松、静脉注射环磷酰胺冲击治疗及新鲜冰冻血浆。8周后,fVIII抑制剂检测呈阴性。

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