Recent Updates in Langerhans Cell Histiocytosis
10.15264/cpho.2015.22.1.15
- Author:
Kyung Nam KOH
1
;
Ho Joon IM
;
Jong Jin SEO
Author Information
1. Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital, Seoul, Korea. pedkkn@gmail.com
- Publication Type:Clinical Trial
- Keywords:
Langerhans cell histiocytosis;
Pathogenesis;
Treatment
- MeSH:
Histiocytes;
Histiocytosis, Langerhans-Cell
- From:Clinical Pediatric Hematology-Oncology
2015;22(1):15-21
- CountryRepublic of Korea
- Language:Korean
-
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.
