Thrombotic microangiopathies: an update

Abstract

Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Although several disease states may manifest as TMA, the two most relevant conditions associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), characterized by prominent brain or renal lesions, respectively. However, occasionally the clinical distinction between these two conditions can be difficult. In this review, we focus on the epidemiologic and diagnostic criteria as well as on the most recent insights into the pathophysiology and treatment of these two conditions.