Open AccessThrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent
Author(s) -
Eun Mi Yang,
Dong Han,
Hee Jo Baek,
Myung Geun Shin,
Young Ok Kim,
Hoon Kook,
Tai Ju Hwang
Publication year - 2010
Publication title -
korean journal of pediatrics/korean journal of pediatrics
Language(s) - English
Resource type - Journals
eISSN - 2092-7258
pISSN - 1738-1061
DOI - 10.3345/kjp.2010.53.3.428
Subject(s) - medicine , adamts , thrombotic thrombocytopenic purpura , pediatrics , gastroenterology , platelet , metalloproteinase , thrombospondin , matrix metalloproteinase
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