Acquired haemophilia (AH) is a rare, often severe bleeding disorder characterised by autoantibodies to coagulation factor VIII (FVIII). Observational studies offer crucial insight into the disease and its treatment. Recombinant activated factor VII (rFVIIa, eptacog alfa activated) was available on an emergency and compassionate use basis from 1988 to 1999 at sites in Europe and North America. In 1996, rFVIIa was approved in Europe for the treatment of AH; it was licensed for this indication in the United States in 2006. Recombinant activated FVII is approved for first-line treatment of bleeding episodes and prevention of bleeding in surgical/invasive procedures in patients with AH. This review provides an up-to-date summary of the haemostatic efficacy of rFVIIa in patients with AH, from the first emergency and compassionate use programmes, to patient registries and a post-marketing surveillance study. In acute bleeding episodes, rFVIIa provided high and consistent rates of control, and available data showed that acute bleed control rates were higher for first-line rFVIIa versus salvage rFVIIa. In surgical procedures, rFVIIa also provided high rates of control. In patients with AH, rFVIIa has a high rate of haemostatic efficacy in acute and surgical bleeding episodes.

中文翻译：

---

重组活化因子VII在获得性血友病患者中的应用。

获得性血友病（AH）是一种罕见的，经常为严重的出血性疾病，其特征在于对凝血因子VIII（FVIII）的自身抗体。观察性研究提供了对该疾病及其治疗的关键见解。1988年至1999年间，在欧洲和北美洲的一些地点可以紧急和富有同情心地使用重组激活的VII因子（rFVIIa，被激活的eptacog alfa）。1996年，rFVIIa在欧洲被批准用于治疗AH。它已于2006年在美国获得该适应症的许可。重组活化FVII被批准用于AH患者的出血事件的一线治疗和外科手术/侵入​​性手术中的出血预防。这篇综述提供了rFVIIa在AH患者中的止血功效的最新摘要，来自第一个紧急和有同情心的使用计划，进行患者登记和售后监测研究。在急性出血发作中，rFVIIa提供了较高且一致的控制率，而现有数据表明，一线rFVIIa的急性出血控制率高于抢救性rFVIIa。在外科手术中，rFVIIa还提供了较高的控制率。在AH患者中，rFVIIa在急性和外科手术出血事件中具有很高的止血功效。