PB1064 - Management of surgery with recombinant porcine factor VIII in acquired hemophilia A

Background: Acquired hemophilia A (AHA) is caused by autoantibodies against factor VIII (FVIII). Surgery can result in uncontrolled bleeding in patients with AHA and is strongly discouraged until hemostasis is secured. Sometimes, surgery has already been performed in referring hospitals for life-/limb-threatening bleeds or before the diagnosis was made, and additional surgery may be inevitable to manage wound bleeds, tissue damage, or other complications. Recombinant porcine FVIII (rpFVIII) is licensed for acute bleeding in AHA, but limited data is available to support its use for surgical prophylaxis.

Aims: To report comprehensive center experience with rpFVIII in the management of surgery in patients with AHA.

Methods: This retrospective study enrolled consecutive patients with confirmed diagnosis of AHA , who received rpFVIII for major emergency surgery. According to center policy, rpFVIII was used if cross-reactive anti-rpFVIII inhibitors were excluded by laboratory testing or were deemed unlikely because of low anti-human FVIII inhibitor. rpFVIII was administered by bolus dosing or continuous infusion and closely monitored by one-stage clot assay.

Results: Ten patients with AHA (4 female, 6 male, median age 60 years) were included. Two patients with pregnancy-related AHA received emergency hysterectomy. Five patients underwent trauma surgery (decompression, wound revision, negative pressure wound therapy), and two underwent laparotomy (one of them twice, for retroperitoneal bleeding and splenectomy). One patient received coronary artery bypass grafting, aortic valve replacement, and mitral valve reconstruction. FVIII monitoring indicated hemostatic efficacy and clinical assessment confirmed good or excellent efficacy in all cases. No thromboembolic events were recorded and all patients recovered from the initial trauma or surgery.

Conclusion(s): rpFVIII provided effective hemostasis in AHA patients undergoing major surgery. Although surgery may still pose significant risks to patients with AHA, potentially life-saving surgical interventions appear feasible using rpFVIII.