Clin Pediatr Hematol Oncol.  2015 Apr;22(1):15-21. 10.15264/cpho.2015.22.1.15.

Recent Updates in Langerhans Cell Histiocytosis

• Koh KN
• Im HJ
• Seo JJ

• KMID: 2168915
• DOI: http://doi.org/10.15264/cpho.2015.22.1.15

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.

Keyword

Langerhans cell histiocytosis; Pathogenesis; Treatment

MeSH Terms

Histiocytes
Histiocytosis, Langerhans-Cell*