Postpartum-acquired hemophilia: A challenging disease to manage

Abstract

Acquired hemophilia is a severe bleeding diathesis that affects both males and females. It is caused by suddenly appearing autoantibodies that interfere with the coagulation factor VIII activity. This disorder is characterized by spontaneous and post-traumatic subcutaneous bleeds and massive mucosal hemorrhages. We report a case of postpartum-acquired hemophilia with a compartment syndrome of right (Rt.) forearm in a 29-year-old woman. Hemostatic tests indicated a prolonged activated partial thromboplastin time (APTT) to 99 s (normal 30.4–40 s), the prothrombin time was 7.8 s (normal 12–14 s), and the platelet count was 271,000 (normal 155,000–450,000). Further workup demonstrated the presence of autoantibody against factor VIII in a titer of 15 Bethesda Units/ml (BU/ml) and a decreased factor VIII activity to 0.04 IU/ml (normal 0.6–1.5 IU/ml). Immunosuppressive treatment with oral prednisone at 60 mg/24 h was initiated, but she was refractory to steroids; therefore, rituximab was added on a weekly basis for 4 weeks. At the same time, factor eight inhibitor bypassing activity (FEIBA), an anti-inhibitor coagulant complex, has been used as a bypassing agent. Reduction of the factor VIII inhibitor titer to 0 BU/ml and an increase of factor VIII activity to 0.38 were achieved. This led to normalization of hemostatic parameters (APTT 38.9 s, factor VIII activity 0.38).

Keywords

• Acquired hemophilia
• hemophilia
• postpartum