Abstract: Thrombotic thrombocytopenic purpura （TTP） is a rare life-threatening disorder. It is characterized by microangiopathic hemolytic anemia，progressive thrombocytopenia，central nervous system disorder，fever and acute renal failure. It is caused by the absent or severe deficiency of the von Willebrand factor-cleaving protease named ADAMTS13. The pathogenesis of TTP is unknown，usually triggered after pregnancy. It causes maternal morbidity and fetal loss. Prompt aggressive management should include timely termination of pregnancy，plasma transfusion and/or plasma exchange.

Key words: Purpura, thrombotic thrombocytopenic, Pregnancy complications, hematologic, Plasma exchange, ADAMTS13