Thrombotic thrombocytopenic purpura (TTP) is a medical emergency characterized by thrombocytopenia associated with microangiopathic hemolytic anemia and organ damage due to visceral ischemia, resulting from deficiency of the enzyme ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13); there should be strong suspicion for this disorder due to its high lethality rate in case of delay in the initiation of therapy [3]. This evidence concerns the gene ADAMTS13 and thrombotic thrombocytopenic purpura.