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Atypical Postoperative Bleeding as a First Manifestation of Acquired Hemophilia A: A Case Report
Maciej Michalak1, Agnieszka Piróg1, Paweł Andruszkiewicz1
12nd Department of Anaesthesiology and Intensive Care, Medical University of Warsaw, Poland.
Acquired hemophilia A (AHA) is a rare autoimmune coagulopathy disorder characterized by the development of inhibitory autoantibodies against coagulation factor VIII. It often presents with spontaneous or post-procedural bleeding in individuals without prior bleeding history and requires prompt recognition and management. We report the case of a 70-year-old male who developed life-threatening hemorrhagic complications following pancreaticoduodenectomy. Re-laparotomy revealed dehiscence of the pancreaticojejunostomy, which was resected, and a temporary Wirsungostomy was created. The patient was admitted to the Intensive Care Unit (ICU) with progressive anemia, subcutaneous hematomas, and recurrent gastrointestinal bleeding. Laboratory tests revealed isolated activated partial thromboplastin time (aPTT) prolongation, lack of correction in the mixing study, reduced factor VIII activity (15.3%), and the presence of an inhibitor (0.7 Bethesda Units; BU). Based on the above, acquired hemophilia A was diagnosed. Treatment included recombinant activated factor VII (rFVIIa) for 6 days and corticosteroid therapy with methylprednisolone. Clinical improvement and nearcomplete remission were achieved, with normalization of aPTT, restoration of factor VIII activity (99.6%), and persistence of only trace inhibitor levels. This case highlights the diagnostic complexity of atypical postoperative bleeding, underscoring the need for a broad differential diagnosis and a high index of suspicion for rare disorders like acquired hemophilia A to ensure timely, life-saving intervention.
Acquired hemophilia A (AHA) is a rare autoimmune coagulopathy disorder characterized by the development of inhibitory autoantibodies against coagulation factor VIII. It often presents with spontaneous or post-procedural bleeding in individuals without prior bleeding history and requires prompt recognition and management. We report the case of a 70-year-old male who developed life-threatening hemorrhagic complications following pancreaticoduodenectomy. Re-laparotomy revealed dehiscence of the pancreaticojejunostomy, which was resected, and a temporary Wirsungostomy was created. The patient was admitted to the Intensive Care Unit (ICU) with progressive anemia, subcutaneous hematomas, and recurrent gastrointestinal bleeding. Laboratory tests revealed isolated activated partial thromboplastin time (aPTT) prolongation, lack of correction in the mixing study, reduced factor VIII activity (15.3%), and the presence of an inhibitor (0.7 Bethesda Units; BU). Based on the above, acquired hemophilia A was diagnosed. Treatment included recombinant activated factor VII (rFVIIa) for 6 days and corticosteroid therapy with methylprednisolone. Clinical improvement and nearcomplete remission were achieved, with normalization of aPTT, restoration of factor VIII activity (99.6%), and persistence of only trace inhibitor levels. This case highlights the diagnostic complexity of atypical postoperative bleeding, underscoring the need for a broad differential diagnosis and a high index of suspicion for rare disorders like acquired hemophilia A to ensure timely, life-saving intervention.
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