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Recent advances in the understanding of the molecular pathogenesis and targeted therapy options in Langerhans cell histiocytosis

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Abstract
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder caused by the clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in the lesion. Advances in genomic sequencing techniques have improved our understanding of the pathophysiology of LCH. Activation of the mitogen-activated protein kinase (MAPK) pathway is a key molecular mechanism involved in the development of LCH.
Recurrent BRAF mutations and MAP2K1 mutations are the major molecular alterations involved in the activation of the MAPK pathway. Recent studies have supported the “misguided myeloid differentiation model” of LCH, where the extent of disease is defined by the differentiation stage of the cell in which the activating somatic MAPK mutation occurs, suggesting LCH. Several studies have advocated the efficacy of targeted therapy using BRAF inhibitors with a high response rate, especially in patients with high-risk or refractory LCH. However, the optimal treatment scheme for children remains unclear.
This review outlines recent advances in LCH, focusing on understanding the molecular pathophysiology, emerging targeted therapy options, and their clinical implications.
Author(s)
강성한고경남김혜리서진경임호준
Issued Date
2021
Type
Article
Keyword
Langerhans cell histiocytosisTherapeuticsPathology
DOI
10.5045/br.2021.2021013
URI
https://oak.ulsan.ac.kr/handle/2021.oak/8002
https://ulsan-primo.hosted.exlibrisgroup.com/primo-explore/fulldisplay?docid=TN_cdi_nrf_kci_oai_kci_go_kr_ARTI_9780098&context=PC&vid=ULSAN&lang=ko_KR&search_scope=default_scope&adaptor=primo_central_multiple_fe&tab=default_tab&query=any,contains,Recent%20advances%20in%20the%20understanding%20of%20the%20molecular%20pathogenesis%20and%20targeted%20therapy%20options%20in%20Langerhans%20cell%20histiocytosis&offset=0&pcAvailability=true
Publisher
Blood Research
Location
대한민국
Language
한국어
ISSN
2287-979X
Citation Volume
56
Citation Number
S1
Citation Start Page
65
Citation End Page
69
Appears in Collections:
Medicine > Medicine
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