Absolute deficiency of ADAMTS-13 leads to diffuse microvascular occlusion due to high molecular weight VWF multimers which promote platelet aggregation and microthrombi formation; therefore, low levels of ADAMTS-13 are of increasing interest in thrombotic-microangiopathies and those clinical conditions like sepsis in which liver failure, as well as other organ dysfunctions are frequently observed [21,22]. Here, ADAMTS13 is linked to thrombotic microangiopathy.