Abstract
INTRODUCTION: Haemophilia A and B are hereditary bleeding disorders that require multidisciplinary perioperative management. Data on orthopaedic surgery outcomes with extended-half-life (EHL) recombinant Fc-fusion factor VIII (rFVIIIFc) and factor IX (rFIXFc) products remain limited. AIMS: To evaluate the efficacy of EHL rFVIIIFc or rFIXFc in major orthopaedic surgery, focusing on haemostasis, safety and factor consumption. METHODS: This prospective study involved persons with haemophilia A or B treated with rFVIIIFc or rFIXFc undergoing orthopaedic surgery. RESULTS: Twenty major orthopaedic surgeries (2018-2023) were included in 19 persons with severe or moderate haemophilia A (n = 14) or B (n = 5), median age 46 years (range 26-60). Procedures included arthroplasty, arthrodesis, arthroscopic synovectomy, prosthetic revision of the knee or ankle, and removal of a femur fracture fixation device. Median hospital stay was 7 days (range 2-18). Median cumulative factor consumption was 300 and 388 IU/kg for haemophilia A and B, respectively. Haemostatic efficacy was rated as 'good' in 95% (n = 18) of cases, 'poor' in 5% (n = 1), and not documented in one case. Median haemoglobin (Hb) change was -2.0 g/dL (range -4.6 to +0.5); no transfusions were required. Complications were reported in 45% (n = 9) of cases (anaemia 40%; blood loss 5%) and managed with oral supplementation of iron and folates. No adverse events related to rFVIIIFc or rFIXFc administration were observed. CONCLUSION: RFVIIIFc and rFIXFc provide effective haemostasis during orthopaedic surgery in patients with haemophilia A and B, with a favourable safety profile. Further multicentre studies are warranted to confirm these results and refine perioperative management guidelines.