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Thrombotic Microangiopathies: Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS). Morphological Features, Differential Diagnosis, and Pathogenesis. Review Article


Authors: E. Honsová
Authors‘ workplace: Pracoviště klinické a transplantační patologie IKEM, Praha
Published in: Čes.-slov. Patol., 44, 2008, No. 3, p. 54-58
Category: Reviews Article

Overview

Thrombotic microangiopathies are characterized by microvascular thrombosis associated with hemolytic anemia, thrombocytopenia, and red blood cell fragmentation. They represent a heterogeneous group of diseases of different etiologies and the entire group shares the same morphological features. Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura are classically associated with thrombotic microangiopathy and the differential diagnosis between these diseases has been very difficult being based more often on the convictions of various specialists than on precisely defined criteria. Recent studies in the field of biology of the von Willebrand factor and also in the field of alternative complement pathway help us to better understand the pathogenesis of these diseases.

Key words:
TTP – von Willebrand factor – ADAMTS13 – complement – factor H


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