Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy caused by an autoantibody mediated deficiency of ADAMTS-13, the von Willebrand factor (VWF) cleaving protease, resulting in the accumulation of ultra-large VWF multimers that leads to the formation of platelet-rich microthrombi causing end-organ ischemia [1]. This evidence concerns the gene VWF and thrombotic microangiopathy.