Division of Angiology and Haemostasis, University Hospitals of Geneva, Switzerland.
Service and Central Laboratory of Haematology, Lausanne University Hospital, Lausanne, Switzerland.
Swiss Med Wkly. 2020 Dec 18;150:w20422. doi: 10.4414/smw.2020.20422. eCollection 2020 Dec 14.
Emicizumab (Hemlibra®, Hoffmann-La Roche, Switzerland) is now available for haemophilia A patients with or without factor VIII inhibitors. Management of bleeding events and replacement therapy for invasive procedures have to be adapted.
To provide a practical guidance for the management of breakthrough bleeding events and elective or urgent surgery in adult and paediatric patients with haemophilia A without inhibitors treated with emicizumab.
Based on the available literature and the experiences collected from adult and paediatric patients treated in Switzerland, the Working Party on Haemostasis of the Swiss Society of Haematology and the Swiss Haemophilia Network worked together to reach a consensus on the management of bleeding events and invasive procedures.
Minor bleeding events and invasive procedures associated with low bleeding risk can be treated without factor replacement therapy in most cases, whereas major bleeding events and high-risk surgery require additional factor VIII replacement at usual doses, at least for the first days. Emicizumab treatment should be continued throughout the procedure and during the postoperative period. Elective major surgery should be planned according to emicizumab dosing for patients with a once-a-month posology. Of note, so far only few data are available on the management of major bleeds and surgery in patients with haemophilia A treated with emicizumab and this practical guidance will have to be regularly updated with growing experience.  .
依库珠单抗（Hemlibra®，罗氏制药，瑞士）现可用于有或无凝血因子 VIII 抑制剂的血友病 A 患者。必须调整出血事件的管理和侵袭性操作的替代治疗。
为无抑制剂的血友病 A 成年和儿科患者接受依库珠单抗治疗时突破性出血事件和择期或紧急手术的管理提供实用指南。
基于现有文献和瑞士收集的成年和儿科患者的经验，瑞士血液学学会止血工作组和瑞士血友病网络共同合作，就出血事件和侵袭性操作的管理达成共识。
在大多数情况下，轻微出血事件和低出血风险相关的侵袭性操作无需进行因子替代治疗，而重大出血事件和高风险手术需要以通常剂量额外补充凝血因子 VIII，至少在最初几天。整个手术过程中和术后期间都应继续使用依库珠单抗治疗。择期大手术应根据每月一次的治疗方案为患者计划。需要注意的是，目前仅获得了少数关于接受依库珠单抗治疗的血友病 A 患者大出血和手术管理的数据，并且随着经验的积累，本实用指南将需要定期更新。
