ReviewBlood research2021
Recent advances in the understanding of the molecular pathogenesis and targeted therapy options in Langerhans cell histiocytosis.
Review in Blood research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 17 citations in OpenAlex.
- Targeted therapy for Langerhans cell histiocytosis with maxillofacial involvement in 20 children.Orphanet journal of rare diseases · 2026Article
- Perianal Langerhans cell hyperplasia characterized by perianal ulcerative lesions after hemorrhoid surgery: A case report.Medicine · 2025Article
- Unraveling the Genetic Landscape of Langerhans Cell Histiocytosis in Korean Patients: Comprehensive Insights from Mutational Profiles and Clinical Correlations.Cancer research and treatment · 2025Article
- Advances in allogeneic hematopoietic stem cell transplantation for Langerhans cell histiocytosis in children.Frontiers in immunology · 2025Review
- Advancements in the understanding and management of histiocytic neoplasms.Blood research · 2024Review
- Multisystemic recurrent Langerhans cell histiocytosis misdiagnosed with chronic inflammation at the first diagnosis: A case report.World journal of radiology · 2024Article
- Progressive nodular histiocytosis with dramatic response to cobimetinib.JAAD case reports · 2022Article
- Langerhans Cell Histiocytosis Manifests with Acute Severe Hypernatremia during Hospitalization.Case reports in nephrology · 2022Article
- Results of TETimaX Trial of Langerhans Cell Histiocytosis Treatment and Perspectives on the Role of Imatinib Mesylate in the Era of MAPK Signaling.Biomedicines · 2021Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.