Article
The role of von Willebrand factor in thrombotic microangiopathy.
Pediatric nephrology (Berlin, Germany) - 1 Aug 2018
Noone Damien G, Riedl Magdalena, Licht Christoph
Abstract excerpt
Thrombotic microangiopathy (TMA) is caused by thrombus formation in the microvasculature. The disease spectrum of TMA includes, amongst others, thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS). TTP is caused by defective cleavage of von Willebrand factor (VWF), whereas aHUS is caused by overshooting complement activation and subsequent endothelial cell (EC) injury. Despite...
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