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腹腔镜胆囊切除术后获得性 A 型血友病严重病例。

Severe case of postpartum-acquired haemophilia A after laparoscopic cholecystectomy.

机构信息

Department of Surgery, Prisma Health Upstate, Greenville, South Carolina, USA.

Department of Surgery, Prisma Health Upstate, Greenville, South Carolina, USA

出版信息

BMJ Case Rep. 2024 Mar 29;17(3):e258812. doi: 10.1136/bcr-2023-258812.

Abstract

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.

摘要

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。

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