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Original Article

Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent

Eun Mi Yang, Dong Kyun Han, Hee Jo Baek, Young Ok Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang

Clin Exp Pediatr. 2010;53(3):428-431. Published online March 15, 2010

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other...

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