Thrombotic thrombocytopenia purpura (TTP), defined clinically by microangiopathic hemolytic anemia and thrombocytopenia, is attributed to either inherited or acquired loss of VWF multimer size regulation caused by severe ADAMTS13 deficiency, resulting in the accumulation of UL–VWF multimers. Here, ADAMTS13 is linked to congenital thrombotic thrombocytopenic purpura.