ArticleThe Journal of experimental medicine2014
BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups.
Article in The Journal of experimental medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 193 papers, 2 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
193 citing papers in PubMed, 2 syntheses or guidelines pooled it, 432 citations in OpenAlex.
- Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review.Oral and maxillofacial surgery · 2021Pooled it
- Merkel cell polyomavirus and Langerhans cell neoplasm.Cell communication and signaling : CCS · 2018Pooled it
- Phase II study of vemurafenib in children and young adults with tumors harboring BRAF V600 mutations: NCI-COG pediatric MATCH trial (APEC1621) Arm G.The oncologist · 2024Trial
- Trial
- Target and Agent Prioritization for the Children's Oncology Group-National Cancer Institute Pediatric MATCH Trial.Journal of the National Cancer Institute · 2017Trial
- Trial
- Langerhans cell histiocytosis: molecular mechanisms underlying pathogenesis and emerging targeted therapeutics.Cancer metastasis reviews · 2026Review
- Class 3 MAP2K1 mutations associate with relapse and multiple treatment lines in multisystem Langerhans cell histiocytosis.Blood advances · 2026Article
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- Watchful waiting in children with unifocal osseous Langerhans cell histiocytosis.The oncologist · 2026Article
- Pathogenic myeloid phenotypes drive disease pathology in a novel human neurohistiocytosis model.Blood · 2026Article
- A Rare Soft Tissue Presentation of Mixed Histiocytosis with Multiple Disseminated Subcutaneous Masses.Acta dermato-venereologica · 2026Article
- Clinicopathological features of gastric Langerhans cell histiocytosis and a literature review.Journal of clinical and experimental hematopathology : JCEH · 2026Review
- Dabrafenib or trametinib in pediatric Langerhans cell histiocytosis with bone lesions: a retrospective efficacy and safety study.BMC cancer · 2026Article
- Targeted therapy for Langerhans cell histiocytosis with maxillofacial involvement in 20 children.Orphanet journal of rare diseases · 2026Article
- Blood MMP-7 and TNF-α levels as potential prognostic biomarkers for adult pulmonary Langerhans cell histiocytosis.Scientific reports · 2026Article
- Histiocytoses and reactive proliferations of histiocytes: current state of the art and evolving concepts-a report from the joint CSHP-EA4HP-SH workshop 2024, Hefei, China.Virchows Archiv : an international journal of pathology · 2026Review
- RAF-independent MEK mutations drive refractory histiocytic neoplasms but respond to ERK inhibition.Cancer cell · 2026Article
- Prognostic factor analysis of oral and maxillofacial Langerhans cell histiocytosis based on clinical findings and tumour microenvironment.BMC oral health · 2026Article
- Langerhans cell histiocytosis manifesting at birth: a neonatal case with BRAF V600E mutation.Diagnostic pathology · 2025Article
133 more citing papers are in PubMed but not listed here.
Corrections and comments
- Erratum issued
Authors and funding
24 authors at 11 institutions in 5 countries.
Funding
Abstract
Langerhans cell histiocytosis (LCH) is a clonal disorder with elusive etiology, characterized by the accumulation of CD207(+) dendritic cells (DCs) in inflammatory lesions. Recurrent BRAF-V600E mutations have been reported in LCH. In this study, lesions from 100 patients were genotyped, and 64% carried the BRAF-V600E mutation within infiltrating CD207(+) DCs. BRAF-V600E expression in tissue DCs did not define specific clinical risk groups but was associated with increased risk of recurrence. Strikingly, we found that patients with active, high-risk LCH also carried BRAF-V600E in circulating CD11c(+) and CD14(+) fractions and in bone marrow (BM) CD34(+) hematopoietic cell progenitors, whereas the mutation was restricted to lesional CD207(+) DC in low-risk LCH patients. Importantly, BRAF-V600E expression in DCs was sufficient to drive LCH-like disease in mice. Consistent with our findings in humans, expression of BRAF-V600E in BM DC progenitors recapitulated many features of the human high-risk LCH, whereas BRAF-V600E expression in differentiated DCs more closely resembled low-risk LCH. We therefore propose classification of LCH as a myeloid neoplasia and hypothesize that high-risk LCH arises from somatic mutation of a hematopoietic progenitor, whereas low-risk disease arises from somatic mutation of tissue-restricted precursor DCs.
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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.