【摘要】 目的提高对获得性血友病 A 的认识。方法报道2例自身免疫病并发获得性血友病 A 患者的临床表现、实验室检查、诊治经过及预后,并复习相关文献。结果 2例患者除原发病即原发性干燥综合征和类风湿关节炎的表现外,均出现自发性大出血,FⅧ凝血活性(FⅧ：C)下降,FⅧ抗体(FⅧ：Ab)水平升高,经糖皮质激素、FⅧ浓缩物等治疗,出血症状均得到控制。结论获得性血友病 A 可并发于自身免疫病,大多数病人表现为突然发生的自发性出血,严重者需要免疫抑制剂、FⅧ浓缩物、凝血酶原复合物等多种药物联合治疗才能控制。更多还原

【Abstract】 Objective To further understand aquired hemophilia A.Methods Report 2 patients with aquired hemophilia A following autoimmune disease,including their clinical manifestations,laboratory exami- nations,courses of diagnosis and therapy and prognosises,and review the correlative literatures.Results Be- sides the manifestations of primary diseases,which were primary Sjogren’s syndrome and rheumatoid arthri- tis,bursting out bleeding was the most prominent character of the 2 patients.FⅧ:C descended and the level of FⅧ:Ab rised.By the therapy of glucocorticoid,FⅧ condensate,etc,the bleeding were all controlled. Conclusion Aquired hemophilia A can follow autoimmune disease.Most of patients burst out bleeding.The severer cann’t be controlled until many kinds of medicines are combined to treat them,which are immunosup- pressor,FⅧ condensate,prothrombin complex,etc.更多还原