Severe ADAMTS13 deficiency leads to formation of the pathological lesion termed thrombotic microangiopathy (TMA) (Benz and Amann, 2009) which affects renal function due to occluded glomerular capillaries containing VWF-rich microthrombi (Manea et al., 2007). The gene discussed is ADAMTS13; the disease is Genetic thrombotic microangiopathy.