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http://dx.doi.org/10.3345/kjp.2010.53.3.428

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

Yang, Eun Mi (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Han, Dong Kyun (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Baek, Hee Jo (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Shin, Myung Geun (Department of Laboratory Medicine, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Kim, Young Ok (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Kook, Hoon (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Hwang, Tae Ju (Department of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
Publication Information
Clinical and Experimental Pediatrics / v.53, no.3, 2010 , pp. 428-431 More about this Journal
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;
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