National Coagulation Centre, St James's Hospital, Dublin, Ireland.
School of Medicine, Trinity College Dublin, Dublin, Ireland.
Haemophilia. 2021 Nov;27(6):e690-e697. doi: 10.1111/hae.14426. Epub 2021 Oct 6.
Recombinant factor IX fusion protein concentrate (rFIXFc) is increasingly used for prophylaxis in people with haemophilia B (PWHB), but experience in the perioperative setting is limited.
To evaluate real-world perioperative factor usage, bleeding and complications in PWHB (≥18 years) who received rFIXFc for surgical haemostasis and to describe the treatment regimens used.
Single centre, retrospective review of all PWHB who underwent a major or minor surgical procedure between June 2017 and July 2020 and received rFIXFc perioperatively for maintenance of surgical haemostasis.
A total of 56 PWHB (45 male and 11 female), including people with mild (n = 32), moderate (n = 4) and severe (n = 20) haemophilia B, underwent 11 major and 131 minor procedures with rFIXFc for surgical haemostasis. Haemostasis was rated as excellent (9/11) or good (2/11) in all major procedures. Median total rFIXFc consumption for orthopaedic surgeries was 972 IU/kg (range 812-1031 IU/kg) and for other major (non-orthopaedic) surgeries was 323 IU/kg (range 167-760 IU/kg). The median number of perioperative rFIXFc infusions was 19 (range 17-26) for orthopaedic surgery and 7 (range 5-17) for other major surgeries. The number of infusions in the postoperative period was determined by procedure and patient factors. Complications included bowel ileus and wound infection. Most minor procedures were managed with single infusion of rFIXFc, with no bleeding complications in 95% of minor procedures. There were no thromboembolic events or inhibitor formation.
This unique data provides real-world evidence that rFIXFc is safe and effective in achieving haemostasis in PWHB undergoing surgery.
重组凝血因子 IX 融合蛋白浓缩物（rFIXFc）越来越多地用于预防乙型血友病（PWHB）患者，但在围手术期的应用经验有限。
评估 PWHB（≥18 岁）在接受 rFIXFc 进行手术止血时的围手术期实际因子使用情况、出血和并发症，并描述使用的治疗方案。
回顾性分析 2017 年 6 月至 2020 年 7 月期间在我院接受重大或小手术且围手术期接受 rFIXFc 以维持手术止血的所有 PWHB 的单一中心病例。
共 56 例 PWHB（45 名男性和 11 名女性），包括轻度（n=32）、中度（n=4）和重度（n=20）乙型血友病患者，共接受 11 例重大手术和 131 例小手术以接受 rFIXFc 进行手术止血。所有重大手术的止血效果均评为优秀（9/11）或良好（2/11）。骨科手术的 rFIXFc 总消耗量中位数为 972IU/kg（范围 812-1031IU/kg），其他重大（非骨科）手术为 323IU/kg（范围 167-760IU/kg）。骨科手术的 rFIXFc 输注中位数为 19 次（范围 17-26 次），其他重大手术为 7 次（范围 5-17 次）。输注次数由手术和患者因素决定。并发症包括肠粘连和伤口感染。大多数小手术只需单次输注 rFIXFc 即可治疗，95%的小手术无出血并发症。没有血栓栓塞事件或抑制剂形成。
本研究提供了真实世界的数据，证明 rFIXFc 安全有效，可用于接受手术的 PWHB 实现止血。
