Four-Year Emicizumab Treatment in an Elderly Patient With Acquired Hemophilia A: A Case Report on Perioperative Management Along With a Literature Review.

Acquired hemophilia A (AHA) is a life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). While immunosuppressive therapy (IST) can eradicate these autoantibodies, it may fail or cause adverse events, especially in elderly patients. Clinical trials involving AHA patients have confirmed the efficiency of emicizumab, a bispecific antibody mimicking FVIII widely used in congenital hemophilia A with or without inhibitors, but the long-term safety and effectiveness in perioperative hemostatic management remain unclear. These issues are important due to the high risk of thrombosis in AHA patients. This report details four years of continuous use of emicizumab and perioperative management in an elderly woman in her 90s with IST-resistant/intolerant AHA. She was diagnosed with AHA one year before the initiation of emicizumab. After initially responding to steroid pulse therapy with a tentative fall in FVIII autoantibodies, she experienced recurrences and repetitive episodes of massive cutaneous bleeding within a year during corticosteroid tapering, which required continuous high-dose steroid therapy. Emicizumab was initiated via enrollment in the AGEHA clinical trial. During the four years of continuous use (the longest reported duration), no major bleeding or adverse events were recorded. The patient underwent emergency surgery for appendicitis, during which hemostasis was maintained with recombinant activated factor VII (rFVIIa). Despite minimal intraoperative blood loss, prolonged drain-site bleeding occurred, possibly due to low emicizumab levels indicated by FVIII activity measured by chromogenic assay. rFVIIa was tapered gradually to postoperative day 27 without thrombotic complications. This case highlights the utility of emicizumab in IST-resistant AHA patients by achieving effective hemostasis and safety against IST-related toxicity. It also demonstrates the beneficial effects of rFVIIa in hemostasis during surgery, emphasizing the importance of careful monitoring in balancing bleeding and thrombotic risks.