Abstract
People with hemophilia A have a total or partial deficiency of factor (F)VIII, causing spontaneous and/or traumatic bleeding into the joints, muscles, and soft tissues. Major surgery may be required to restore joint mobility or treat the symptoms of common comorbidities in people with hemophilia A. Additional factor replacement is recommended during the perioperative period; collated information on the experience of emicizumab-treated people with hemophilia A during major surgery is currently lacking. To provide a consolidated narrative summary of the experience with emicizumab in people with hemophilia A without FVIII inhibitors undergoing major surgery, a comprehensive literature search was performed using PubMed/MEDLINE (cut-off date: March 31, 2024); the abstract books for applicable congresses (2016-2024) were searched manually. Studies were included if reporting original data on people with hemophilia A of all ages and hemophilia A severities without FVIII inhibitors on emicizumab prophylaxis who had undergone major surgery. Outcomes collected included perioperative surgical management, adverse events, and bleeding events. Twenty publications were included; 72 procedures were reported. Twenty-two orthopedic and 34 other major procedures were specifically described. FVIII replacement was used to manage 66 procedures perioperatively, and 25 procedures were managed in conjunction with antifibrinolytics. Fifteen procedures resulted in a bleeding event, and one individual experienced a thrombotic event. No deaths were reported. This review provides a consolidated narrative of the currently reported experiences of emicizumab-treated people with hemophilia A without FVIII inhibitors undergoing major surgery, helping to support the future management decisions of emicizumab-treated people with hemophilia A during surgery.