Case series of thrombotic thrombocytopenic purpura in children and adolescents

Thrombotic thrombocytopenic purpura (TTP) is a well-described entity in adults but is rarely observed in children. The authors describe a series of seven children with suspected acquired TTP. Clinical findings included petechiae, purpura, or jaundice ( 6), central nervous system events ( 5), fever ( 3), diarrhea ( 3), renal insufficiency ( 2), and hematuria ( 2). Significant central nervous system events included cerebral vascular accidents ( 2), altered mental status ( 2), seizures ( 1), and hemiparesis ( 1). Patients were treated with daily plasma infusions (1/7) or plasma exchange (5/7). Response was prompt, although relapses were frequent. Decreased vWF-protease activity was found in four of five cases and vWF-protease inhibitors were found in three of five cases. Although rare, TTP is a life-threatening illness that does occur in children and should be considered in the differential diagnosis of thrombocytopenia with hemolytic anemia.