Thrombotic microangiopathies: an update.
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.
Franchini
Massimo
M
Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliera di Verona, Verona, Italy. mfranchini@mail.univr.it
D051722:ADAM Proteins / Q000172:deficiency / Q000276:immunology / Q000502:physiology / Q000627:therapeutic use; D000071120:ADAMTS13 Protein; D000328:Adult; D019090:Case Management; D002648:Child; D004927:Escherichia coli Infections / Q000150:complications; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D011994:Recombinant Proteins / Q000627:therapeutic use
D011994:Recombinant Proteins; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human