ArticleOrphanet journal of rare diseases2023
Clinical features, genomic profiling, and outcomes of adult patients with unifocal Langerhans cell histiocytosis.
Article in Orphanet journal of rare diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 7 citations in OpenAlex.
- Application of pretreatmentAnnals of hematology · 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
- Langerhans Cell Histiocytosis With Hypothalamic-pituitary and Bone Involvement: A Report of 2 Cases.JCEM case reports · 2025Article
- [A case of adult temporal bone langerhans cell histiocytosis presenting as posterior canal dehiscence syndrome and literature review].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2025Review
- The Impact of Hybrid Therapy on Langerhans Cell Histiocytosis of the Mandible in an Older Male: A Case Report and Literature Review.Case reports in otolaryngology · 2025Article
- Article
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundLangerhans cell histiocytosis (LCH) is a rare highly heterogeneous histiocytosis, which can be divided into single system and multiple system disease according to site of involvement. There is a paucity of studies examining unifocal LCH in adults in the molecular era.
resultsWe retrospectively analysed records from 70 patients with unifocal LCH. The median age at diagnosis was 36 years (18-69). The most common organ involved was the bone (70.0%), followed by pituitary gland (7.1%). Target gene sequencing of lesion tissues was performed on 32 of the 70 patients. MAPK/PI3K pathway alterations were observed in 78.1% of the patients; the most common mutations included BRAF
conclusionsIn our large cohort of adults with unifocal LCH, we found that prognosis of unifocal LCH in adults was very good, and age < 30 years at diagnosis was associated with increased relapse risk.
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