Systemic Lupus Erythematosus Presenting as Hematoma of the Hand Due to Acquired Inhibitors to Factor VIII: Early and Prolonged Remission Achieved with Upfront Rituximab

摘要

Acquired hemophilia is a rare autoimmune disorder that is a result of antibodies against clotting factor VIII and it presents with excessive or prolonged bleeding, often into the muscles. Thrombotic phenomena with lupus anticoagulant are common in patients with systemic lupus erythematosus (SLE). We report a rare case of a young female with no significant past medical history presenting with hematoma of the hand who was later on found to have acquired hemophilia, SLE with antiphospholipid antibodies (APLA). She was successfully treated with upfront rituximab and prednisolone leading to early and prolonged remission. No increased incidence of infections was noted. Upfront rituximab appears to be a safe and effective option in the management of such patients when compared to use of cytotoxic agents such as cyclophosphamide; however, further data from randomized studies is needed. Neutropenia and acquired hemophilia should also be considered to be listed under hematological manifestations of SLE diagnostic criteria, as they are not uncommon in such patients.