ArticleInternational journal of hematology2020
Clinical features and outcomes of adult Langerhans cell histiocytosis: a single-center experience.
Article in International journal of hematology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Enigmatic Case of Adult-Onset Langerhans Cell Histiocytosis with Aggressive Bony Involvement.Journal of Brown hospital medicine · 2026Article
- Insights into etiology and management of a case of Langerhans cell histiocytosis manifesting as perianal lesions: a case report.Journal of medical case reports · 2025Article
- Recurrent multisystem Langerhans cell histiocytosis involving the female genitalia: A case report.World journal of clinical cases · 2024Article
- The value of 18F-FDG PET/CT in Langerhans cell histiocytosis.Annals of nuclear medicine · 2024Article
- Somatic ARAF mutations in pediatric Langerhans cell histiocytosis: clinicopathologic, genetic and functional profiling.Clinical and experimental medicine · 2023Article
- Clinical and prognostic features of Langerhans cell histiocytosis in adults.Cancer science · 2023Article
- Adult Onset Langerhans Cell Histiocytosis: Clinical Characteristics and Treatment Outcomes.Clinical hematology international · 2023Article
- Role ofQuantitative imaging in medicine and surgery · 2022Article
- Frequency detection of BRAF V600E mutation in a cohort of pediatric langerhans cell histiocytosis patients by next-generation sequencing.Orphanet journal of rare diseases · 2021Article
- Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study.Medicina (Kaunas, Lithuania) · 2021Article
- Gastrointestinal lesion in adult-onset Langerhans cell histiocytosis.International journal of clinical oncology · 2020Article
- [18F]FDG- PET/CT in Diagnosis, Staging, and Management of Patients with Langerhans Cell Histiocytosis.Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaArticle
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Authors and funding
7 authors.
Funding
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Abstract
Langerhans cell histiocytosis (LCH) is a clonally expanding neoplasm characterized by the accumulation of CD1a + CD207 + myeloid dendritic cells. As LCH is a rare disease and is presumed to mainly affect children, the clinical features and treatment outcomes of adult LCH have been poorly documented. We retrospectively reviewed 53 adult patients with LCH who were referred to the Institute of Medical Science, the University of Tokyo from 2005 to 2018. The median age at diagnosis was 42 years with a slight female predominance (57%). The time between onset and diagnosis varied among patients (median, 8 months; range, 0-144 months). In total, 40% of the patients had single organ involvement and 60% had multiple organ involvement. Overall, the most frequently affected organ was bone (62%), followed by the central nervous system (34%), and the lung (28%). Twenty-six patients required systemic treatment, and 25 patients underwent the Special C regimen. Twenty patients (80%) who underwent Special C regimen showed a partial response or better with favorable toxicity. All but one patient is still alive. Median progression-free survival has not been reached despite a median follow-up of 35.5 months. Immunohistochemistry revealed that 39% of patients were positive for BRAF-V600E, which was a lower proportion than in previous reports from North America and Europe.
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