All issues > Volume 53(3); 2010

Original Article

Korean J Pediatr. 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 EM Yang¹, Dong Kyun DK Han¹, Hee Jo HJ Baek¹, Young Ok YO Kim¹, Myung Geun MG Shin², Hoon H Kook¹, Tai Ju TJ 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

Correspondence Hoon H Kook ,Email: hoonkook@chonnam.ac.kr

Received: November 12, 2009; Revised: January 26, 2010   Accepted: February 18, 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.

Keywords :Purpura, Thrombotic thrombocytopenic, ADAMTS13 protein