Acquired Haemophilia in a Patient with Systemic Lupus Erythematosus

Acquired haemophilia is a rare bleeding disorder caused by production of autoantibodies directed against coagulation factor VIII (FVIII), with high morbidity and mortality. Systemic lupus erythematosus (SLE) is one of the known associated underlying conditions. We report a case of life threatening bleeding in a patient with acquired haemophilia caused by FVIII inhibitor and underlying SLE. The patient presented with spontaneous bruises over right calf and ankle. Coagulation studies showed isolated prolonged activated partial thromboplastin time, reduced FVIII level, and high FVIII inhibitors. The coagulopathy did not improve despite pulse methylprednisolone followed by oral steroid, azathioprine and intravenous immunoglobulin. One and a half year later, she developed massive, life-threatening haemorrhage refractory to pulse cyclophosphamide, corticosteroid, and plasmapheresis. Finally, combination therapy with rituximab, cyclosporine A, cyclophosphamide, and corticosteroid resulted in clinical and biological remission.