Thrombotic thrombocytopenic purpura in autoimmune hepatitis

A 19-year-old woman presented with clinical manifestations of sudden, fulmi nant thrombotic thrombocytopenic purpura associated with autoimmune hepatit is and autoimmune thrombocytopenic purpura. Although thrombotic thrombocyto penic purpura may, rarely, be associated with systemic lupus erythematosus and other autoimmune diseases, to our knowledge, the syndrome has never bee n described in association with autoimmune hepatitis. In this patient, too, the etiology of thrombotic thrombocytopenic purpura associated with autoim mune disease remains elusive. The patient was treated with corticosteroid, which brought about no improvement in her condition, and she died of multio rgan failure. Diagnosis is challenging, but prompt diagnosis is necessary b ecause thrombotic thrombocytopenic purpura is a life-threatening syndrome w hose prognosis has been improved significantly by early plasmapheresis trea tment.