PMID: 18184524Jan 11, 2008Paper

Thrombotic microangiopathy in a 17-year-old patient: TTP, HUS or a bit of both?

Clinical Nephrology
J GerthG Wolf

Abstract

Thrombotic microangiopathies are characterized by the development of hyaline thrombi in small vessels resulting in thrombocytopenia, microangiopathic hemolysis, and organ dysfunction. Thrombotic-thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are two major clinical syndromes of thrombotic microangiopathies. Although differential diagnosis between TTP and HUS is commonly determined in the clinical setting, recent evidence suggests major pathophysiological differences between the two diseases. Autoimmune inhibitors or genetic mutations of a von Willebrand factor (VWF) cleaving metalloprotease (ADAMTS13) leads to the accumulation of unusually large multimeric forms of VWF in TTP, facilitating adherence of platelets and development of microthrombi. In contrast, classic HUS is caused by infection with verocytotoxin-producing bacteria. This toxin induces endothelial injury, apoptosis and inflammation. Endothelial injury results in increased shear-stress, fostering cleavage of VWF, but thrombosis eventually develops. One would assume that measurement of ADAMTS13 activity and/or detection of verocytotoxin could easily contribute to the differential diagnosis of TTP or HUS. We report on a case of a young patient with ...Continue Reading

Citations

Dec 17, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sybille PelrasJérôme Harambat
Sep 30, 2010·Medizinische Klinik·Regina HermannGunter Wolf

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