Deficiency in ADAMTS13 results in TTP, a disorder of thrombotic microangiopathy, characterized by an abnormal persistence of UL-VWF multimers.36Similar to the pathology in systemic inflammation/sepsis, thrombotic microangiopathies such as TTP are associated with acute dysfunctional endothelial cell activation, indicated by up to fourfold increased VWF antigen (VWFAg) and VWF propeptide (VWFpp).37 The gene discussed is VWF; the disease is thrombotic thrombocytopenic purpura.