ArticleClinical hematology international2023
Adult Onset Langerhans Cell Histiocytosis: Clinical Characteristics and Treatment Outcomes.
Article in Clinical hematology international, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 4 citations in OpenAlex.
- Clinical features, diagnostic difficulties and therapeutic enlightenment of adult-onset multisystem Langerhans cell histiocytosis complicated with panhypopituitarism and central diabetes insipidus: a case report and systematic literature review.Frontiers in oncology · 2026Article
- A case report of delayed-diagnosed adult multisystem Langerhans cell histiocytosis with isolated MAP2K1 somatic mutation and systematic literature review.Frontiers in oncology · 2026Article
- 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
- A Rare Convergence: Adult-Onset Langerhans Cell Histiocytosis and HIV Infection.European journal of case reports in internal medicine · 2023Article
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeLangerhans cell histiocytosis (LCH) is a rare disease that can affect all tissues and organs. Our study evaluated the clinical characteristics and treatment outcomes of adult-onset LCH patients in a tertiary center. MATERIALS AND
methodsAdult patients diagnosed with LCH were retrospectively evaluated. Their initial symptoms, stratification according to disease involvement, treatment details, treatment responses, and overall and progression-free survival (PFS) were analyzed.
resultsThirty-three patients were included. There were 21 single system LCH, 10 multisystem LCH, and 2 pulmonary LCH patients. Patients with single system unifocal involvement were successfully treated with local therapies such as surgery and radiotherapy. Most of the multisystem LCH patients and patients with single system multifocal involvement were treated with systemic chemotherapy. Cladribine was the first choice in 10 out of 11 patients who received chemotherapy. Among all patients, the overall response rate (ORR) was 97%. Among those who had cladribine in the first-line the ORR was 81%. All these patients achieved a complete remission and were alive at the last visit. The median follow-up was 38 (range, 2-183) months. The median PFS has not yet been reached. Ten-year PFS was 90.9%.
conclusionBesides successful local treatments with surgery and radiotherapy, our study provides information for front-line cladribine treatment.
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