In this condition, patients develop a secondary thrombotic microangiopathy associated with a decreased activity of a protease (disintegrin and metalloproteinase with thrombospondin motifs 13, ADAMTS-13), leading to increased circulating ultra-large von Willebrand factor units (vWF), platelet overconsumption and organ failure secondary to vWF-rich microvascular thromboses. Here, VWF is linked to thrombotic microangiopathy.