CHU Lille, Université de Lille, Lille, France.
Département d'Hématologie-Hémostase, Hôpital Universitaire de Tours, Tours, France.
Haemophilia. 2019 Sep;25(5):731-737. doi: 10.1111/hae.13817. Epub 2019 Jul 11.
Emicizumab (Hemlibra ) recently became available and requires an adaptation for managing bleeding, suspected bleeding and emergency or scheduled invasive procedures in haemophilia A patients with inhibitor. This implicates a multidisciplinary approach and redaction of recommendations for care that must be regularly adapted to the available data.
The following text aims to provide a guide for the management of people with haemophilia A with inhibitor treated with emicizumab in case of bleeding or invasives procedures.
The French network on inherited bleeding disorders (MHEMO), the French Reference Centre on Haemophilia (CRH), in collaboration with the French Working Group on Perioperative Haemostasis (GIHP) have been working together to make proposals for the management of these situations.
Haemostatic treatment and other medications should be given stepwise, according to the severity and location of the bleeding or the risk of bleeding of the procedure as well as the haemostatic response obtained at each step in order to ensure an optimal benefit/risk ratio.
The lack of data means that it is only possible to issue proposals rather than recommendations.
艾美赛珠单抗（Hemlibra）最近已上市，需要对其进行调整，以便在有抑制剂的血友病 A 患者中管理出血、疑似出血以及紧急或计划进行的侵入性操作。这需要采取多学科方法，并对护理建议进行修订，这些建议必须定期适应现有数据。
本文旨在为接受艾美赛珠单抗治疗的有抑制剂的血友病 A 患者在出血或侵入性操作时提供管理指南。
法国遗传性出血性疾病网络（MHEMO）、法国血友病参考中心（CRH）与法国围手术期止血工作组（GIHP）合作，提出了这些情况下的管理建议。
应根据出血的严重程度和部位或操作的出血风险以及在每一步中获得的止血反应，逐步给予止血治疗和其他药物，以确保最佳的获益/风险比。
由于缺乏数据，目前只能提出建议而非推荐意见。
