Abstract / Summary
Background/Objectives: Acquired hemophilia A (AHA) is caused by neutralizing factor VIII (FVIII) autoantibodies and is exceptionally rare in children. Pediatric treatment evidence is limited, and recommended hemostatic agents may be inaccessible. Case: An 11-year-old previously healthy girl presented with soft-tissue, mucosal, and genitourinary bleeding, an isolated markedly prolonged activated partial thromboplastin time, FVIII activity of approximately 1.4–2%, and an FVIII inhibitor. Recombinant activated factor VII was financially inaccessible to the family, and activated prothrombin complex concentrate (APCC) was unavailable. After a multidisciplinary discussion with the family, non-activated four-factor prothrombin complex concentrate (4F-PCC) was administered off-label; corticosteroid-based treatment was initiated to eradicate the inhibitor. Results: Gross hematuria resolved during the treatment period, and microscopic hematuria cleared subsequently. By July 2025, FVIII activity was 125%, the inhibitor was undetectable, immunosuppression had been withdrawn, and no recurrent major AHA-related bleeding had been reported. Conclusions: This case illustrates individualized decision-making in pediatric AHA when recommended hemostatic agents cannot be implemented. The temporal association between 4F-PCC administration and bleeding resolution does not establish efficacy, safety, or equivalence to APCC.