Department of Surgery, Prisma Health Upstate, Greenville, South Carolina, USA.
Department of Surgery, Prisma Health Upstate, Greenville, South Carolina, USA
BMJ Case Rep. 2024 Mar 29;17(3):e258812. doi: 10.1136/bcr-2023-258812.
Acquired factor VIII inhibitor, also known as acquired haemophilia A, has been associated with the postpartum state in young females. Treatment of acquired haemophilia A is focused on two goals: control of bleeding and eliminating the factor VIII inhibitor. Management requires successful intervention to accomplish both goals. Here, we describe the presentation and management of a case of acquired haemophilia A resulting in particularly severe and protracted intra-abdominal bleeding after routine laparoscopic cholecystectomy in a young and otherwise healthy female at 3 months postpartum. Due to diffuse intra-abdominal bleeding, she required return to the operating room on five occasions for intra-abdominal packing, reassessment of bleeding and ultimate fascial closure. Her abdomen was open for 5 days. She was treated with activated recombinant human factor VIIa to bypass inhibited factor VIII, and with immunosuppression using steroids, cyclophosphamide and anti-CD20 monoclonal antibody rituximab. She achieved remission after 6 weeks of treatment.
获得性因子 VIII 抑制剂,也称为获得性血友病 A,与年轻女性的产后状态有关。获得性血友病 A 的治疗重点是两个目标:控制出血和消除因子 VIII 抑制剂。管理需要成功干预才能实现这两个目标。在这里,我们描述了一例获得性血友病 A 的表现和管理,一名年轻且健康的女性在产后 3 个月接受常规腹腔镜胆囊切除术,随后发生特别严重和持久的腹腔内出血。由于弥漫性腹腔内出血,她需要返回手术室 5 次进行腹腔内填塞、重新评估出血和最终筋膜闭合。她的腹部敞开了 5 天。她接受了激活的重组人因子 VIIa 治疗以绕过抑制的因子 VIII,并接受了类固醇、环磷酰胺和抗 CD20 单克隆抗体利妥昔单抗的免疫抑制治疗。她在治疗 6 周后达到缓解。