ReviewCancer science2018
Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment.
Review in Cancer science, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 80 papers.
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Who cites it
80 citing papers in PubMed, 171 citations in OpenAlex.
- Phase 2 study of oral thalidomide-cyclophosphamide-dexamethasone for recurrent/refractory adult Langerhans cell histiocytosis.Leukemia · 2022Trial
- A case of neonatal Langerhans cell histiocytosis initially presenting with haemorrhagic vesicles: a case report and literature review.BMC pediatrics · 2026Review
- Pulmonary Langerhans Cell Histiocytosis Presenting as Multiple Obstructing Bronchial Lesions and Pulmonary Masses.The clinical respiratory journal · 2026Article
- Adult-Onset Multisystem Langerhans Cell Histiocytosis: Atypical Skeletal and Endocrine Manifestations.Cureus · 2026Article
- Prognostic factor analysis of oral and maxillofacial Langerhans cell histiocytosis based on clinical findings and tumour microenvironment.BMC oral health · 2026Article
- Orbital Langerhans Cell Histiocytosis in Young Pediatric Patients: Two Case Reports and Literature Review.Pediatric health, medicine and therapeutics · 2026Article
- Eosinophilic granuloma: Case-report of spontaneous resolution.Radiology case reports · 2025Article
- Spontaneous Regression of Adult Multi-system Langerhans Cell Histiocytosis Presenting as Liver Tumor Rupture.Internal medicine (Tokyo, Japan) · 2025Review
- Article
- Adult Spinal Langerhans Cell Histiocytosis with Conservative Treatment: A Case Report.Spine surgery and related research · 2025Article
- Article
- Adult onset multisystem Langerhans cell histiocytosis initially presenting with arginine vasopressin deficiency: a case report.Frontiers in medicine · 2025Article
- Diagnostic challenges in pulmonary Langerhans cell histiocytosis: A case report of nodular presentation in an adult.Respiratory medicine case reports · 2025Article
- Case report: Pulmonary non-Langerhans cell histiocytosis in a dog with acute interstitial granulomatous pneumonia.Frontiers in veterinary science · 2025Article
- Article
- Posterior Instrumentation without Curettage Promotes Rapid Restoration of Adult Spinal Langerhans Cell Histiocytosis.Spine surgery and related research · 2024Article
- Anti-LGI1 encephalitis and Langerhans cell histiocytosis: Two associated entities? A case report.Heliyon · 2024Article
- Current State of Targeted Therapy in Adult Langerhans Cell Histiocytosis and Erdheim-Chester Disease.Targeted oncology · 2024Review
- Challenges in diagnosis and management of Langerhans Cell Histiocytosis in a 13-month-old child: a rare case report.Annals of medicine and surgery (2012) · 2024Article
- Diffuse large B-cell lymphoma withClinical case reports · 2024Article
20 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by the accumulation of CD1a+/Langerin+ LCH cells and wide-ranging organ involvement. Langerhans cell histiocytosis was formerly referred to as histiocytosis X, until it was renamed in 1987. Langerhans cell histiocytosis β was named for its morphological similarity to skin Langerhans cells. Studies have shown that LCH cells originate from myeloid dendritic cells rather than skin Langerhans cells. There has been significant debate regarding whether LCH should be defined as an immune disorder or a neoplasm. A breakthrough in understanding the pathogenesis of LCH occurred in 2010 when a gain-of-function mutation in BRAF (V600E) was identified in more than half of LCH patient samples. Studies have since reported that 100% of LCH cases show ERK phosphorylation, indicating that LCH is likely to be a clonally expanding myeloid neoplasm. Langerhans cell histiocytosis is now defined as an inflammatory myeloid neoplasm in the revised 2016 Histiocyte Society classification. Randomized trials and novel approaches have led to improved outcomes for pediatric patients, but no well-defined treatments for adult patients have been developed to date. Although LCH is not fatal in all cases, delayed diagnosis or treatment can result in serious impairment of organ function and decreased quality of life. This study summarizes recent advances in the pathophysiology and treatment of adult LCH, to raise awareness of this "orphan disease".
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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.