Laparoscopic Cholecystectomy for Acute Cholecystitis in a Patient with Acquired Hemophilia A: A Case Report of Perioperative Hemostatic Management with Plasma Exchange.

introductionAcquired hemophilia A (AHA) is a rare but potentially life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Surgery in patients with AHA is challenging because of the substantial risk of severe perioperative hemorrhage. However, no consensus has been reached on the optimal perioperative hemostatic strategies, especially for urgent surgical settings that require rapid correction of coagulation function, because of the rarity of this condition. CASE PRESENTATION: A 79-year-old woman was diagnosed with AHA and received immunosuppressive therapy (prednisolone and cyclophosphamide) and hemostatic treatment (activated prothrombin complex concentrate). She developed acute cholecystitis on day 23. However, surgery was initially contraindicated due to inadequate coagulation control, persistently prolonged activated partial thromboplastin time, low FVIII activity, and high FVIII inhibitor titer. Antibiotic therapy was initiated, and endoscopic nasobiliary gallbladder drainage was attempted on day 26. However, the procedure was unsuccessful. The worsening abdominal pain on day 27 prompted further interventions. Plasma exchange (PE) was performed to rapidly reduce the FVIII inhibitor titers and correct the coagulation status before laparoscopic cholecystectomy. The coagulation parameters markedly improved after 3 PE sessions, as evidenced by the shortened activated partial thromboplastin time, increased FVIII activity, and significant reduction in FVIII inhibitor titers. This enabled a safe laparoscopic cholecystectomy with minimal intraoperative blood loss on day 30. No additional PE was required, and the patient was discharged uneventfully on POD 16. The FVIII inhibitor was eliminated within 2 months, and immunosuppressive therapy was discontinued 4 months after surgery. The patient has been in remission to date.

conclusionsThis case highlights the potential utility of PE as a bridging strategy to achieve rapid hemostatic correction in patients with AHA requiring urgent surgery, especially those with high-titer FVIII inhibitors. Immunosuppressive therapy and bypassing agents remain the cornerstones of AHA management. However, PE may be an effective adjunct treatment option for refractory cases or in time-constrained settings. This report provides valuable insights into individualized multidisciplinary approaches that may facilitate safe surgical intervention in patients with AHA.