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Review Article
Recent Updates in Langerhans Cell Histiocytosis
Clin Pediatr Hematol Oncol 2015;22:15-21.
Published online April 30, 2015
© 2015 Korean Society of Pediatric Hematology-Oncology

Kyung-Nam Koh, M.D., Ho Joon Im, M.D. and Jong Jin Seo, M.D.

Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children’s Hospital, Seoul, Korea
Correspondence to: Kyung-Nam Koh
Department of Pediatrics, Asan Medical
Center Children’s Hospital, 88,
Olympic-ro 43-gil, Songpa-gu, Seoul
138-736, Korea
Tel: +82-2-3010-5994
Fax: +82-2-473-3725
E-mail: pedkkn@gmail.com
Received April 1, 2015; Revised April 15, 2015; Accepted April 16, 2015.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Langerhans cell histiocytosis (LCH) encompasses a wide range of clinical presentation and its clinical course varies widely from spontaneous regression to severe disseminated disease with the risk of permanent consequences. Although pathogenesis of LCH has been a conundrum, recent advances have led to a better understanding of the molecular pathogenesis of the disease. Especially, advanced genomic analyses have suggested that LCH is a disorder of MAPK pathway mutations. Optimal treatment for LCH has not been established yet. Histiocyte Society has launched a new international multi-center clinical trial, LCH-IV to solve remaining major issues in the treatment of LCH. The aim of this review is to provide an overview of recent understandings on the pathogenesis and treatment of LCH.
Keywords: Langerhans cell histiocytosis, Pathogenesis, Treatment
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