Patients whose ADAMTS13 activity is ≥ 10% are outside the scope of these guidelines, because such patients do not meet the criteria for designated intractable/rare diseases defined by the Japanese Ministry of Health, Labour, and Welfare (MHLW) (ADAMTS13 activity < 10% for TTP). The gene discussed is ADAMTS13; the disease is thrombotic thrombocytopenic purpura.