[Thrombotic thrombocytopenic purpura]

Abstract

Distinguishing thrombotic thrombocytopenic purpura (TTP) from other thrombotic microangiopathies requires measurement of ADAMTS13 enzyme activity, but treatment must often be commenced before results from the ADAMTS13 analysis is available. Scoring systems to facilitate prediction of severe ADAMTS13 deficiency and therefore immediate clinical management have been developed. This combined with advances in treatment and monitoring holds optimism for improvements in late effects and survival in future patients. In this review, we discuss status in diagnosing, managing, and follow-up of patients with TTP.