Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.

Kremer Hovinga, Johanna Anna; Heeb, Silvan Rolf; Skowronska, Magdalena; Schaller Tschan, Monica Maria (2018). Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Journal of thrombosis and haemostasis, 16(4), pp. 618-629. Wiley-Blackwell 10.1111/jth.13956

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Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end-organ damage. The latter particularly affects the brain, the heart, and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although their clinical presentations often overlap, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS-13 deficiency, either immune-mediated as a result of circulating autoantibodies, or caused by mutations in ADAMTS-13. HUS develops following an infection with Shiga-toxin producing bacteria, or as the result of excessive activation of the alternative pathway of the complement system because of mutations in genes encoding complement system proteins.

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