Acquired Hemophilia A (AHA): A Rare Bleeding Disorder In a Tertiary Care Hospital In Dhaka, Bangladesh
DOI:
https://doi.org/10.3329/jom.v25i2.74380Keywords:
Acquired Hemophilia A, factor VIII, autoantibodies, factor VIII inhibitors.Abstract
Acquired Hemophilia A (AHA) is a rare bleeding disorder. This results from autoantibodies to factor VIII (FVIII) that neutralizes the ability of factor VIII. This causes potentially severe bleeding episodes that carries a high risk of morbidity and mortality. Acquired Hemophilia usually occurs over 60 years of age or in postpartum period and associated with other autoimmune conditions. The diagnosis should be suspected when patients with new onset of bleeding without prior personal or family history of bleeding. The hallmark of this condition is mucocutaneous bleeding leading to ecchymosis, malena, hematoma, hematuria and spontaneous oozing from wounds. Management should uphold both hemostatic therapies to minimize bleeding and immune modulation strategies to re-establish immune tolerance to factor VIII. Here we present an older male case of such Acquired Hemophilia A without history of previous anticoagulant therapy as well as family history or personal history of bleeding episodes. The patient has got hemiarthroplasty with prosthesis with bone cementing due to fracture neck of femur. After 10th post-operative day he was diagnosed with clot retention in catheter in-situ and oozing from wound site after 12th post-operative day. His laboratory reports showed prolongation of Prothrombin Time (PT) and Activated Partial Prothrombin Time (APTT). His factor VIII was very low level and factor VIII inhibitor showed the time depended inhibitor presence. Thus diagnosed as Acquired Hemophilia A (AHA), the rare case in Bangladesh. He was managed with medications that resulted in normalization of factor VIII level.
J MEDICINE 2024; 25: 173-175
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