获得性血友病A(AHA)是由靶向凝血因子VIII(FVIII)的自身抗体(抑制剂)产生引起的出血性疾病。它的特点是突然发作,常引起软组织大量严重出血。当凝血测试显示PT正常时,诊断为获得性血友病A,延长APTT,FVIII活性降低,正常的VWF活动,和阳性FVIII抑制剂。止血治疗主要包括旁路治疗,激活外源性凝血途径,绕过FVIII或因子IX的需要。Emicizumab,替代FVIII功能的双特异性抗体,可用于防止出血。免疫抑制治疗对于抑制或根除抑制剂是必要的。大多数患者通过治疗进入缓解期,但有些人死于与免疫抑制治疗相关的出血症状或感染。
Acquired hemophilia A (AHA) is a bleeding disorder caused by autoantibody (inhibitor) production targeting blood coagulation factor VIII (FVIII). It is characterized by sudden onset, and often causes extensive and severe bleeding in soft tissue. Acquired hemophilia A is diagnosed when coagulation tests show normal PT, prolonged APTT, decreased FVIII activity, normal VWF activity, and positive FVIII inhibitor. Hemostatic therapy mainly consists of bypass therapy, which activates the extrinsic coagulation pathway, bypassing the need for FVIII or factor IX. Emicizumab, a bispecific antibody that substitutes for FVIII function, can be used to prevent bleeding. Immunosuppressive therapy is necessary to suppress or eradicate inhibitors. The majority of patients go into remission with treatment, but some die from bleeding symptoms or infections associated with immunosuppressive therapy.