Rezaieyazdi Zahra, Sharifi-Doloui Davood, Hashemzadeh Kamila, Shirdel Abbas, Mansouritorghabeh Hassan
Rheumatic Disease Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Blood Coagul Fibrinolysis. 2011 Dec;22(8):738-41. doi: 10.1097/MBC.0b013e32834a5c8e.
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.
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。
