CO-89 | FEMORAL FRACTURE SURGERY IN AN ELDERLY, FRAIL PATIENT WITH ACQUIRED HEMOPHILIA A RECEIVING EMICIZUMAB PROPHYLAXIS: A CASE REPORT

C. Simion^1|2, E. Zanon^1|2, B. Stocco^1|2, F. Sartorello¹, C. Bulato¹, G. Saggiorato¹, P. Simioni^1|2 | ¹First Chair of Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, Padova University Hospital, Italy; ²Hemophilia Center, Padova University Hospital, Italy

Background. Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder predominantly affecting elderly and frail patients. Although emicizumab was developed for congenital hemophilia A, its use has progressively expanded to selected patients with AHA. However, evidence supporting perioperative management during major surgery remains scarce. We describe the clinical course and perioperative management of an elderly woman with AHA who underwent urgent femoral fracture surgery while receiving emicizumab prophylaxis.
Case Report. An 80-year-old woman with AHA experienced recurrent spontaneous hematomas and laboratory instability despite treatment with prednisone and cyclophosphamide. FVIII activity fluctuated widely (<1%–66%), while inhibitor levels progressively increased, reaching 37 BU/mL. Due to refractory disease, cumulative immunosuppressive toxicity, and severe clinical frailty (body weight 29 kg), emicizumab prophylaxis was initiated using an individualized reduced regimen consisting of a single 90 mg dose followed by 60 mg every two weeks, without a loading phase. This approach resulted in clinical stabilization. Emicizumab plasma concentrations reached 10.4 µg/mL after 3 weeks and remained stable at 11.0 µg/mL after 5 weeks. In September 2025, the patient sustained a femoral fracture requiring urgent orthopedic surgery. Emicizumab prophylaxis was continued, and perioperative hemostatic management was based on recombinant activated factor VII (rFVIIa, 90 µg/kg per bolus; 3 mg per dose) administered according to a predefined tapering protocol over seven days, for a total cumulative dose of 96 mg. Tranexamic acid (500 mg three times daily) was added on postoperative day 3 because of anemia. Although a postoperative decline in hemoglobin required transfusion support with packed red blood cells, the patient remained hemodynamically stable, with no major bleeding, hematoma formation, or thrombotic complications. At the day 10 follow-up, hemoglobin was 11.6 g/dL. A laboratory relapse occurred in January 2026 without clinical bleeding.
Conclusions. This case illustrates that major orthopedic surgery can be successfully performed in selected elderly and frail patients with AHA receiving emicizumab prophylaxis, using individualized dosing strategies and structured rFVIIa-based perioperative management. In the absence of specific recommendations for this clinical scenario, detailed case reporting may help inform clinical decision-making in high-risk patients.