VWF and vascular disorder: The pathophysiology of these late complications is thought to rely on both the sequelae of microvascular thrombosis during the acute phase and also a subnormal, non-severe, chronic ADAMTS13 deficiency, with an accumulation of hyper-adhesive, ultra-large VWF multimers released from the endothelium, leading to subclinical vasculopathy and cumulative vascular injury [51].