ADAMTS13 and congenital thrombotic thrombocytopenic purpura: TTP is a rare and life-threatening diffuse thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ damage (such as kidneys, central nervous system, etc) caused by microthrombosis, which is its main pathological feature.[3] TTP is associated with severe defects in ADAMTS13.[4] The first acute episode of TTP usually occurs in adulthood, and the main cause is anti-ADAMTS13 autoimmune disease.