Coagulation Disorders Unit, Department of Haematology, Helsinki University Hospital Comprehensive Cancer Centre and University of Helsinki, Helsinki, Finland.
Coagulation Centre, Department of Medicine/Section of Haematology and Coagulation, Sahlgrenska University Hospital and Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Haemophilia. 2022 Sep;28(5):713-719. doi: 10.1111/hae.14585. Epub 2022 May 16.
Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients.
To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway.
Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based on local practises.
Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose for major HA surgeries (n = 8) was 48 IU/kg (range: 35-57), with total consumption up to Day 14 of 427 IU/kg (196-568). For the two major HB surgeries (in one patient), preoperative rFIXFc doses were 50 IU/kg and 20 IU/kg; total consumption up to Day 14 was 130 IU/kg and 40 IU/kg. Median preoperative rFVIIIFc/rFIXFc bolus doses for minor HA (n = 10) and HB (n = 4) surgeries were 50 IU/kg (24-79) and 47 IU/kg (40-71), with total consumption up to Day 5 of 138 IU/kg (49-404) and 100 IU/kg (43-125), respectively. Intraoperative and postoperative haemostatic responses were rated as at least good/excellent for 24/25 surgeries, with bleeding episodes reported in only three surgeries.
Nordic real-world experiences suggest that EHL products can be used safely and effectively for peri-operative haemostasis. Further research is required to develop local dosing guidelines for optimised treatment schedules.
北欧血友病治疗中心（HTC）之间围手术期剂量推荐方案存在差异，适用于血友病 A/B（HA/HB）患者的延长半衰期（EHL）因子浓缩物。
总结北欧四个 HTC 的临床记录中的回顾性数据，描述使用 EHL 重组因子 VIII/IX Fc（rFVIIIFc/rFIXFc）融合蛋白的真实世界手术经验。
对接受手术且接受 rFVIIIFc/rFIXFc 治疗的 HA/HB 患者记录因子剂量和手术结果。围手术期因子剂量方案由临床医生根据当地实践确定。
20 名患者共进行了 25 次手术，除了一次小 HA 手术外，其余手术均采用推注方式；8 次小手术为儿科患者。8 次主要 HA 手术（n=8）的术前 rFVIIIFc 中位剂量为 48 IU/kg（范围：35-57），至第 14 天的总消耗量为 427 IU/kg（196-568）。对于两次主要 HB 手术（在一名患者中），术前 rFIXFc 剂量分别为 50 IU/kg 和 20 IU/kg；至第 14 天的总消耗量分别为 130 IU/kg 和 40 IU/kg。10 次小 HA 和 4 次小 HB 手术的术前 rFVIIIFc/rFIXFc 推注剂量中位数分别为 50 IU/kg（24-79）和 47 IU/kg（40-71），至第 5 天的总消耗量分别为 138 IU/kg（49-404）和 100 IU/kg（43-125）。25 次手术中有 24 次的术中及术后止血反应被评为至少良好/优秀，仅有 3 次手术报告了出血事件。
北欧真实世界经验表明，EHL 产品可安全有效地用于围手术期止血。需要进一步研究以制定当地剂量指南，优化治疗方案。
