Fulminant thrombotic thrombocytopenic purpura in a patient with the limited form of scleroderma: successful outcome using plasma exchange.

Abstract

A 35-year-old woman with a 20-year history of limited scleroderma presented with a 7-day history of low grade fever associated with a red colored urine, 2 days of jaundice and lower extremity petechiae. On admission she had a rapidly deteriorating neurological status associated with thrombocytopenia and microangiopathic anemia. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was made and treatment begun with fresh frozen plasma, plasma exchange and pulse corticosteroids. After a stormy 28-day hospital course she was discharged without residual problems of TTP.