Thrombotic microangiopathies – hemolytic-uremic syndromes and thrombotic thrombocytopenic purpura

Česká verze

Authors: T. Seeman ¹; Ľ. Podracká ²; Š. Štolbová ¹; K. Bláhová ¹
Authors place of work: Pediatrická klinika 2. LF UK a FN Motol, Praha ¹; Detská klinika, Lekárska fakulta Univerzity Komenského a DFNsP, Bratislava ²
Published in the journal: Čes-slov Pediat 2017; 72 (2): 99-108.
Category: Dětská nefrologie - 2.část

Summary

Thrombotic microangiopathies are a heterogeneous group of disease that causes damage of endothelial cells of small arteries in different organs that lead to thrombi formation and organ ischemia. The laboratory markers of TMA are typically a triad of non-immune hemolytic anemia, thrombocytopenia and acute kidney injury. Thrombotic microangiopathies could be differentiated according their etiology to hemolytic-uremic syndromes (HUS) – typical HUS (caused by Shiga-toxin producing E. coli) and atypical HUS (aHUS, caused mainly by dysregulation of alternative pathway of complement) and thrombotic thrombocytopenic purpura (TTP, severe deficiency of von Willebrand factor cleaving protease called ADAMTS13). Treatment of TMA depend on its etiology – only symptomatic therapy in typical HUS (dialysis, transfusion), plasmapheresis, immunosuppression or plasma infusions in TTP and monoclonal anti-C5 antibody eculizumab in aHUS.

KEY WORDS:
thrombotic microangiopathies, thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome

Zdroje

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