[Diagnostic and therapeutic guidelines of thrombotic microangiopathies of the Spanish Apheresis Group]

Abstract

Thrombotic microangiopathies (TMA) are disorders defined by the presence of a microangiopathic hemolytic anemia (with the characteristic hallmark of schistocytes in the peripheral blood smear), thrombocytopenia and organ malfunction of variable intensity. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are the most important forms of TMA and, without the adequate treatment, they are associated with high morbimortality. In recent years, significant advances in the knowledge of the pathophysiology of TMA have occurred. Those advances have allowed us to move from a syndromic diagnosis with a similar treatment to all entities to the search of etiologic diagnosis which would lead to a specific treatment, finally leading to a better outcome of the patient. This document pretends to summarize the current status of knowledge of the pathophysiology of TMA and the therapeutic options available, and to offer a diagnostic and therapeutic practical tool to the professionals caring for the patients.

Keywords: Aféresis; Apheresis; Hemolytic uremic syndrome; Microangiopatía trombótica; Plasma exchange; Púrpura trombocitopénica trombótica; Recambios plasmáticos; Síndrome urémico hemolítico; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura.