Acquired Hemophilia A Associated with Venous Thrombosis and Very High Inhibitor Titer: A Challenging Scenario

Acquired Hemophilia (AH) poses several challenges to clinicians due to potential delays in diagnosis, based on a high index of suspicion, and a high risk of limb and life-threatening bleeding. We here report a case of AH with extremely high inhibitor titer (up to 1200 BU) in a patient who also developed venous thrombosis requiring anticoagulant treatment after prolonged immobilization for femur fracture. Multiple lines of immunosuppressive treatment were needed to achieve inhibitor eradication, probably due to the extremely high inhibitor titer, bleeding management also required several lines of treatment with bypassing agents. Bleeding treatment was here monitored by global hemostatic assays. Management of AH in a reference center allowed to achieve complete remission even in a very intricate condition. was transferred to our unit for transfusion-dependent anemia and persistently prolonged Activated Partial Thromboplastin Time (APTT). The patient was first admitted to a psychiatric unit for a reduction in his mood and attempted suicide; a fracture of the left femur secondary to an accidental fall at home was diagnosed. In consideration of the time since the fall, the patient was managed with conservative therapy with complete immobilization and prophylaxis of venous thromboembolism with Low Molecular Weight Heparin (LMWH). During rehabilitative therapy, painful chest wall and right lower limb muscle hematomas occurred associated with progressive anemia requiring transfusion. The patient was then transferred to an internal medical unit after a CT scan detected active bleeding in the thoracic muscle associated with prolonged APTT for which LMWH was discontinued and fresh frozen plasma administered. He was admitted to our unit 12 days after the first detection of prolonged APTT. The patient presented poor general clinical conditions with a high thrombotic risk due to immobilization for the fracture. A clinical exam revealed a large hematoma of the lower and upper limbs and back. His blood count showed severe anemia. Isolated prolonged APTT (106 seconds) was confirmed. AH was suspected, clotting FVIII activity