PB0006 - Delayed Diagnosis of Acquired Hemophilia A Results in Worse Clinical Outcome

Background: Acquired hemophilia is a rare but potentially life-threatening disease, involving development of autoantibodies directed against plasma coagulation factors. It is crucial to establish an early diagnosis and to initiate an appropriate treatment.

Aims: To present a case of patient with delayed diagnosis of acquired hemophilia A, his treatment and outcome.

Methods: A 66-year-old male was admitted to the hospital with a clinical presentation of a huge hematoma in his left leg and right chest. Both his medical and family history were negative for clotting disorder at any point before. The hematoma in his leg occurred one month earlier and it was treated as a muscle rupture.The blood tests revealed hemoglobin level of 76g/L, WBC 12x10(9)/L, and platelet count 693x10(9)/L Screening hemostasis tests revealed prothrombin and thrombin time in normal range, but significantly prolonged activated partial thromboplastine time to 59s (normal range 26-34s). Factor VIII level was 0.7%. The Bethesda assay confirmed inhibitor titre of 10.2 BU.

Results: The bleeding was treated with a by-passing agent rVIIa (NovoSeven). Immunosuppressive treatment was started with Cyclophosphamide 100mg and Prednison 100mg daily. On the 8-th day of hospitalization NovoSeven was stopped as factor VIII level increased to 6.2% and the inhibitors dropped to 1.1BU. However, the next day a new hemorrhage appeared in both legs and in left arm with a drop in hemoglobin level and development of a hemorrhagic shock which was promptly treated with red blood cell transfusions and other simptomathyc therapy. A new treatment with NovoSeven was initiated again and immunosuppressive treatment continued. The patient was discharged from the hospital on the 25-th day of hospitalization with an ongoing treatment with Cyclophosphamide and Prednison.

Conclusion(s): This case report illustrates a condition with acquired hemophilia A with delayed diagnosis and a life-threatening hemorrhage where the treatment was prolonged and the consummation of factor greatly increased.