Korean J Pediatr.  2010 Mar;53(3):428-431. 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 EM
• Han DK
• Baek HJ
• Shin MG
• Kim YO
• Kook H
• Hwang TJ

• KMID: 2281326
• DOI: http://doi.org/10.3345/kjp.2010.53.3.428

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.

Keyword

Purpura; Thrombotic thrombocytopenic; ADAMTS13 protein

MeSH Terms

Adolescent
Anemia, Hemolytic
Autoantibodies
Child
Endothelial Cells
Humans
Immunoglobulin G
Plasma
Plasma Exchange
Purpura
Purpura, Thrombotic Thrombocytopenic
Steroids
Thrombocytopenia
Thrombotic Microangiopathies
Thymine Nucleotides
Autoantibodies
Immunoglobulin G
Steroids
Thymine Nucleotides