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    Poster Networking

    Hemophilia and Rare Bleeding Disorders

    Poster Networking Session

    PB1236 - Real world experience of perioperative management in people with hemophilia receiving extended half life FVIII and FIX concentrates: A multicentric and cross-sectional study in Northern Spain

    Tuesday, June 27, 2023
    18:30 – 19:30 ET
    Room: Exhibition
    Background: Recombinant extended half-life FVIII (rEHL-FVIII) and FIX (rEHL-FIX) concentrates are increasingly used for prophylaxis in people with hemophilia A (PwHA) and B (PwHB) respectively, but experience in the perioperative setting is limited.

    Aims: To evaluate our real-world experience in adult PwHA/PwHB treated with rEHL-FVIII or rEHL-FIX products for maintaining perioperative haemostasis.

    Methods: Multicentric cross-sectional study and retrospective study was performed between January 2019 and January 2023 in 7 Spanish hospitals. We review all PWHA/PwHB who underwent major surgery o minor surgery/invasive procedures receiving rEHL-FVIII/FIX during perioperative period.

    Results: A total of 39 patients (37 PwHA and 2 PwHB), including mild HA (n = 8), moderate HA (n = 7), severe HA (n = 22) and severe HB (n = 2), underwent 9 major surgeries (3 orthopaedic) and 45 minor procedures. Perisurgical therapy with factor included 34 rFVIII-Fc, 4 rurioctocog-alfa pegol, 6 damoctocog alfa pegol, 8 turoctocog alfa pegol; and 2 rFIX-FP. Haemostasis during surgery was rated as at least good or excellent in most major 7/8 and all minor surgeries. A mild hematoma was reported after 3 major and 1 minor surgeries.
    Median total consumption rEHL-FVIII for major procedures up to day 7 was 270.5 IU/kg (range 83.3–333.3 IU/kg) and 100 IU/kg and 50 UI/kg of rEHL-FIX for two major surgeries in a patient with HB. Median preoperative rEHL-FVIII dose for major HA surgeries was 45 IU/kg (range: 35–50), and 50 IU/kg of rEHL-FIX for both major HB surgeries. Median consumption of rEHL-FVIII for minor procedures up to day 5 was 110.5 IU/kg (range 28.6-307.5 IU/kg) with median preoperative rFVIII doses for minor HA surgeries of 45 IU/kg (28.6–60 IU/kg). No thrombotic events or inhibitor emergence were detected.

    Conclusion(s): Real-world evidences suggest that rEHL-FVIII and rEHL-FIX concentrates are safe and effective for bleed protection in PwHA / PwHB respectively during and after surgery.