UNRAVELING A RARE COMPLICATION: ACQUIRED HEMOPHILIA EMERGING POST-SURGERY

Case Presentation: 70 year old male was admitted for total hip arthroplasty. Post surgery, he experienced an episode of hematemesis leading to drop hb from 14 to 8 mg/dl. EGD showed no evidence of active bleeding. he then started developing hematoma and ecchymosis over his IV and PICC line. A CT scan showed soft tissue hematoma Hematology was consulted. SPEP, Lupus coagulant and mixing studies were ordered to rule out other cause of bleeding. His PTT was elevated with undetectable level of factor VIII and high level of inhibitor. He was initiated on FEIBA infusion along with cyclophosphamide. subsequently, he developed atrial fibrillation with RVR. A repeat CT showed Psoas muscle hematoma. After receiving total 14 units of pRBC with 1 unit of plasma, he opted for palliative care. He was discharged home with hospice care.

Discussion: In hemophilia, APTT is prolonged. Mixing study is conducted to determine whether the clotting time corrected after mixing. If the clotting time fails to correct, it suggests the presence of an inhibitor, which is a hallmark of acquired hemophilia. To confirm diagnosis, Factor VIII activity and an inhibitor level will be checked.• Acquired hemophilia is treated with glucocorticoids, cytotoxic therapy and bypassing agents like FEIBA or rFVIIa• Relapse rate following remission is roughly 20%. With 70% of those who relapsed having a second complete remission.

Conclusions: Acquired hemophilia is very rare autoimmune disorder with annual incidence of 1 to 2 cases per million people, mainly characterized by development of autoantibodies (typically IgG) against factor VIII.In acquired hemophilia, inhibitors bind the specific clotting factor, typically factor VIII, interfering with its function and/or reduce its half-life. These condition can cause abnormal Visceral bleeding including retroperitoneal or muscle hematoma.Acquired hemophilia is mainly found in patient with older age, those with malignancy, other autoimmune conditions ( such as RA, SLE), and pregnancy or postpartum period. It is also associated with certain medications like clopidogrel, omalizumab.Acquired hemophilia is rare but potentially life threatening condition. Early diagnosis and treatment is important to prevent mortality.