Case Presentation: A 21 year old African-American male with no medical history presented with multiple organ injuries secondary to gunshot . He had exploratory laparotomy with repair of abdominal wall , colonic resection, splenectomy, and gastric repair. Two weeks post-surgery , patient developed right femoral vein and bilateral cephalic vein thrombosis . Therapeutic dose of enoxaparin was started and continued with warfarin until therapeutic INR was achieved , following which the patient started bleeding from multiple mucosal sites with development of abdominal hematoma needing erythrocyte transfusions. Warfarin was held and multiple bags of Fresh Frozen Plasma and Vitamin K were given but the bleeding failed to improve and lab works showed persistently elevated Partial Thromboplastin Time [PTT (>100)] with near normal PT (16) and dropping hemoglobin. Extensive workup revealed absence of lupus anticoagulant and mixing study was significant for partial correction of PTT indicating the presence of an inhibitor. This was followed by measurement of Factor VIII activity which was found to be less than 1% with factor VIII inhibitor level of 12 Bethesda unit. Patient was started on high dose steroids and recombinant factor VIIa leading to immediate improvement in bleeding. He was then started on Rituximab infusions with decreasing factor VIII inhibitor levels and gradual normalization of PTT.
Discussion: Acquired factor VIII deficiency is caused by autoantibody against Factor VIII. Although it generally presents with a classic picture of spontaneous or post surgical muco-cutaneous bleeding , it is a diagnostic menace to physicians because of its extreme rarity , especially when patients are on anticoagulation. As a result, there is often a delay in diagnosing this potentially life threatening condition which presents as a major bleed 87% of the time and with 22% mortality. Elevated PTT with normal PT and INR on a patient with previously normal PTT or no prior history of bleeding diathesis can be a strong clue.
Conclusions: Acquired Hemophilia A is a disease entity with a classic presentation of spontaneous multifocal mucocutaneous bleeding. But due to its rarity, diagnosing this condition can be a challenge particularly in a setting where the patient has been on anticoagulation which further delays the proper treatment of this condition. Therefore, specific lab tests like mixing studies followed by factor VIII activity and Factor VIII inhibitors should be considered in the proper clinical setting as early diagnosis and treatment can prove to be life-saving.