Korean Journal of Pediatrics 2010;53(3):428-431.
Published online March 15, 2010.
Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent
Eun Mi Yang1, Dong Kyun Han1, Hee Jo Baek1, Young Ok Kim1, Myung Geun Shin2, Hoon Kook1, Tai Ju Hwang1
1Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
2Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
청소년기에 발생한 ADAMTS-13 활성도 저하와 항체 양성을 보인 혈전저혈소판혈증자색반병 1례
양은미1, 한동균1, 백희조1, 김영옥1, 신명근2, 국훈1, 황태주1
1전남대학교 의과대학, 화순 전남대학교병원 소아과학교실
2전남대학교 의과대학, 화순 전남대학교병원 진단검사의학과교실
Correspondence: 
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Received: 12 November 2009   • Revised: 26 January 2010   • Accepted: 18 February 2010
Abstract
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 is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.
Key Words: Purpura, Thrombotic thrombocytopenic, ADAMTS13 protein


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