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KMID : 0387820150220010015
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Clinical Pediatric Hematology-Oncology
2015 Volume.22 No. 1 p.15 ~ p.21
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Recent Updates in Langerhans Cell Histiocytosis
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Koh Kyung-Nam
Im Ho-Joon
Seo Jong-Jin
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Abstract
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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.
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KEYWORD
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Langerhans cell histiocytosis, Pathogenesis, Treatment
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