Surgical outcomes in patients with haemophilia A or B receiving extended half-life recombinant factor VIII and IX Fc fusion proteins: Real-world experience in the Nordic countries.

Abstract

IntroductionPerioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half‐life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients.AimTo 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.MethodsFactor 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.ResultsTwenty 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.ConclusionNordic 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.