ArticleCancer2018
CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.
Article in Cancer, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 66 papers.
What it found
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Who cites it
66 citing papers in PubMed, 128 citations in OpenAlex.
- Article
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- Progressive Cerebellar Dysfunction, Pituitary Insufficiency, and Severe Skeletal Fragility in Adult Survivorship of Childhood Multisystem Langerhans Cell Histiocytosis: A Case Report.Clinical case reports · 2026Article
- Pathogenic myeloid phenotypes drive disease pathology in a novel human neurohistiocytosis model.Blood · 2026Article
- Efficacy of MAPK inhibitors in children with neurodegenerative Langerhans cell histiocytosis: Results from the European Consortium for Histiocytosis.HemaSphere · 2026Article
- Progress over perfection: evolving treatments in LCH.Blood advances · 2026Article
- Neurodegenerative Langerhans cell histiocytosis: long-term follow-up of 63 patients from the Italian Registry.Haematologica · 2026Article
- Novel CSF biomarkers for facilitating diagnosis of CNS-HLH from other neuroinflammatory disorders.Blood advances · 2026Article
- "Multifocal, intra-axial Langerhans cell histiocytosis presenting with central diabetes insipidus in teenage pregnancy: A case report".Radiology case reports · 2026Article
- Advances in the diagnosis and management of pediatric Langerhans cell histiocytosis and Rosai-Dorfman disease: therapies, biomarkers, and response assessment.Hematology. American Society of Hematology. Education Program · 2025Review
- Histiocyte Society blueprint for Langerhans cell histiocytosis research: from cell-of-origin to a more comprehensive cure.Haematologica · 2025Review
- Refractory Tumorous and Neurodegenerative Histiocytosis Treated With Intra-Arterial Chemotherapy.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Sustained Response to Pan-BRAF Inhibitor Plixorafenib (FORE8394, PLX8394) in a Young Adult With Neurodegenerative Langerhans Cell Histiocytosis.JCO precision oncology · 2025Article
- Article
- Article
- Disease response criteria in Langerhans cell histiocytosis: a global view.International journal of hematology · 2025Review
- Approach to the Patient: From Endocrinopathy to the Diagnosis of a Histiocytic Disorder.The Journal of clinical endocrinology and metabolism · 2025Review
- Article
- Childhood Langerhans cell histiocytosis hematological involvement: severity associated with BRAFV600E loads.Blood · 2025Article
- BRAFV600E induces key features of LCH in iPSCs with cell type-specific phenotypes and drug responses.Blood · 2025Article
6 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
45 authors at 17 institutions in 3 countries.
Funding
Abstract
backgroundCentral nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH.
methodsCerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Additionally, the presence of BRAFV600E was tested in peripheral mononuclear blood cells (PBMCs) as well as brain biopsies from LCH-ND patients, and the response to BRAF-V600E inhibitor was evaluated in 4 patients with progressive disease.
resultsOsteopontin was the only consistently elevated CSF protein in patients with CNS-LCH compared with patients with other brain pathologies. BRAFV600E DNA was detected in CSF of only 2/20 (10%) cases, both with LCH-ND and active lesions outside the CNS. However, BRAFV600E
conclusionIn LCH-ND patients, BRAFV600E
Indexed as
Identifiers
What OpenQuestion holds
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.