摘要：Thrombotic Thrombocytopenic Purpura (TTP) is a rare acute-onset disease caused by the congenital or acquired deficiency of von Willebrand factor cleaving protease, ADAMTS13, leading to inappropriate microvascular thrombosis and ischemic organ damage. Despite prompt recognition and fast appropriate treatment with plasmaexchange have dramatically improved survival, this thrombotic microangiopathy is still life-threatening, with 10-15% mortality rate in acute phase; in addition, TTP still has a great burden of morbidity, with a big impact on the quality of life of affected patients. New therapeutic agents are currently under evaluation, in particular for resistant and atypical forms, and look promising
