Acquired Hemophilia A: A Case Report and Literature Review on a Diagnostic and Therapeutic Conundrum

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder in which the body spontaneously produces inhibitory antibodies against coagulation factor VIII.This leads to severe, potentially lifethreatening bleeding in patients who have no personal or family history of bleeding problems.The condition carries substantial mortality and remains difficult to diagnose and treat, especially in resource-limited settings.Here, we present three consecutive patients with AHA who were managed at a tertiary hematology center in northern India between 2016 and 2026.Each case illustrates a clinically distinct presentation and a different treatment course.The first patient was a 24-year-old man with pemphigus foliaceus and a history of Castleman disease previously treated with rituximab.Hemostasis was achieved with factor eight inhibitor bypassing activity (FEIBA), an activated prothrombin complex concentrate, but he developed refractory disease, with his inhibitor titer rising more than 20-fold from 16 to 332 Bethesda units (BU) despite first-line prednisolone and cyclophosphamide, after which second-line rituximab was started.Emicizumab was recommended for long-term hemostatic prophylaxis, but the patient was unable to access it due to financial constraints, and he was subsequently lost to follow-up.The second patient was a 35-year-old previously healthy man who presented with a post-traumatic popliteal hematoma and hematuria.Following treatment with prednisolone and cyclophosphamide, the inhibitor became undetectable at eight months, and he was in clinical remission; complete remission could not be confirmed because factor VIII activity was not reassessed.He was subsequently lost to follow-up.The third patient was a 75-year-old woman with longstanding diabetes mellitus and hypertension.She presented with a sudden-onset sublingual hematoma, and high-resolution computed tomography (HRCT) confirmed epiglottic and hypopharyngeal wall thickening causing narrowing of the supraglottic airway.Hemostasis was achieved with FEIBA, alongside prednisolone and subsequent outpatient rituximab, and she was discharged.Approximately four months after discharge, and at least four months after the last FEIBA dose, she suffered an acute myocardial infarction, and her family subsequently chose comfort-focused care.This report underscores the wide clinical variability of AHA, the importance of thorough coagulation workup in any unexplained bleeding episode, the difficulties of managing refractory inhibitor disease in resourcelimited settings, and the critical value of HRCT when evaluating hematomas located near the airway.