Abstract Number: PB0885
Meeting: ISTH 2020 Congress
Background: Emicizumab has revolutionised the management of severe haemophilia A with FVIII inhibitors. The sentinel Haven 1 trial showed an 87% bleed reduction on prophylaxis. Experience in surgery is limited mainly to minor procedures.
Aims: To report the surgical experience in two NZ Haven1 trial patients undergoing major surgical procedures with high dose FVIII and activated FVII (Novoseven) respectively.
Methods: The patients (trial arms A&D) had an elective excision of an expanding anterior thigh pseudotumour (37x7x8cm) causing erosion of the femoral cortex, and acute open laparotomy for an NSAID-induced duodenal ulcer, at trial weeks 114 and 176 repectively.
Results: Neither patient had any other bleeding nor treatment side effects on trial. The pseudotumour predated trial entry by 3 years, required regular aspiration for symptom relief and persisted despite dose escalation to 3mg/kg. The baseline inhibitor 0.6 BU allowed surgery with FVIII 67000 units by c.i. over 14 days without requirement for bypass therapy. Perioperative blood loss was 700mls, drains were removed at 48 hours and peak bovine chromogenic FVIII 319%. The inhibitor titre was 8.9BU on day 15. There were no perioperative complications. Patient 2 (inhibitor 42BU) became hypotensive secondary to haematemesis (Hb58 g/L) and required multiple red cell and platelet transfusions. Bleeding was uncontrollable endoscopically, despite high-dose PPI and TxAc, necessitating early open laparotomy to oversew an H.Pylori negative chronic duodenal ulcer. Novoseven 422mg was administered over 16 days. Regular monitoring for MAHA was negative, there was no recurrence of bleeding (Hb 94 at discharge) and normal midline laparotomy wound healing. Both patients remain well on Emicizumab prophylaxis.
Conclusions: Major surgery can be safely performed in patients on Emicizumab receiving high dose FVIII replacement where the inhibitor can be swamped or using high doses of Novoseven. Neither patient developed microangiopathic haemolysis nor other thrombotic complication despite TxAc and intensive transfusion support.
To cite this abstract in AMA style:Ockelford P, Buyck H, Slavin K, Ramsay B, Devane P, Tan S. Major Surgery in Emicizumab Haven 1 Trial Patients: The New Zealand Experience [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/major-surgery-in-emicizumab-haven-1-trial-patients-the-new-zealand-experience/. Accessed November 29, 2023.
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