Susoctocog alfa for major surgery in acquired Hemophilia A

Abstract Acquired hemophilia A (AHA) is a rare but serious bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII), affecting previously healthy individuals of all ages. At diagnosis, 90–100% of patients present with acute bleeding, often life- or limb-threatening. Surgery is strongly discouraged due to the risk of uncontrollable hemorrhage, and available data on surgical outcomes in AHA are limited, with underreporting of fatal events likely. The current study evaluated the use of susoctocog alfa, a recombinant porcine sequence FVIII (rpFVIII), for perioperative hemostatic management in AHA patients undergoing emergency or elective major surgery. We retrospectively analyzed patients diagnosed with AHA (defined by FVIII activity below normal and inhibitor >0.6 BU/ml) who received at least one dose of rpFVIII prior to or following major surgery. Patients in partial or complete remission at the time of surgery were excluded. Major surgery was defined as procedures involving entry into a body cavity, crossing mesenchymal barriers, or significant alteration of normal anatomy. Fourteen patients (6 female, 8 male; mean age 62 years, range 34–88) underwent 17 major surgeries. Median FVIII activity was 1.8 IU/dl (range <0.5–16), and median inhibitor titer was 7 BU/ml (range 1–467). Cross-reactive anti-rpFVIII inhibitors were assessed in 5 patients, with borderline positivity (0.5 BU/ml) in one. All patients received an initial dose of 200 U/kg rpFVIII, followed by individualized dosing to maintain FVIII activity >80 IU/dl for at least 5 days postoperatively. Surgical procedures included fasciotomy with hematoma evacuation (n=6), laparotomy (n=4), hysterectomy (n=2), and five other diverse interventions. RpFVIII was used as first-line therapy in 4 interventions (including CABG and portacath placement), achieving good or excellent hemostasis without revision surgery. In 13 interventions, rpFVIII was used as rescue therapy—either after failed bypassing agents or delayed AHA diagnosis. Hemostasis improved in all cases, although one patient died from bleeding. Several patients required revision surgery due to tissue damage or open wound management. No thromboembolic events occurred within 30 days. Four patients developed resistance to rpFVIII after 1–3 weeks and were switched to bypassing agents. In conclusion, susoctocog alfa was effective in restoring hemostasis in AHA patients undergoing major surgery. When used as planned, first-line therapy within a specialized center, outcomes were consistently good to excellent. As rescue therapy, rpFVIII was also effective in most life-threatening situations. These findings may inform future guidelines and best practices for managing surgical emergencies in patients with AHA.