ArticleBlood2022
International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults.
Article in Blood, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 157 papers, 4 of them syntheses that pooled it.
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Who cites it
157 citing papers in PubMed, 4 syntheses or guidelines pooled it, 238 citations in OpenAlex.
- Impulsivity, Compulsivity, Social Inappropriateness, and Other Neuropsychiatric Features in Cerebellar Histiocytosis.Cerebellum (London, England) · 2026Pooled it
- Prevalence of oral lesions in Langerhans cell histiocytosis: a systematic review and meta-analysis.Oral and maxillofacial surgery · 2026Pooled it
- Clinical care in interstitial lung disease: a critical appraisal of clinical guidance documents.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- Pulmonary langerhans cell histiocytosis secondary to Marijuana use: a case report and systematic review of the literature.BMC pulmonary medicine · 2025Pooled it
- Clofarabine for relapsed/refractory LCH and non-LC histiocytosis.Blood advances · 2026Trial
- No overshoot of bone turnover after withdrawal of denosumab treatment of adults with Langerhans cell histiocytosis: a prospective clinical trial.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2025Trial
- Phase 2 study using low dose cytarabine for adult patients with newly diagnosed Langerhans cell histiocytosis.Leukemia · 2024Trial
- Multifocal osseous and cutaneous Rosai-Dorfman-Destombes disease presenting as an aggressive intramedullary femoral lesion.Radiology case reports · 2026Article
- A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.Tuberculosis and respiratory diseases · 2026Article
- Review
- Class 3 MAP2K1 mutations associate with relapse and multiple treatment lines in multisystem Langerhans cell histiocytosis.Blood advances · 2026Article
- Vitamin D levels and skeletal quality of life in adult Langerhans cell histiocytosis.Endocrine connections · 2026Article
- Outcome of treatment with trametinib in adults with histiocytic neoplasms in the United Kingdom.British journal of haematology · 2026Article
- A Persistent Flexural Dermatosis Unmasking Adult Langerhans Cell Histiocytosis: A Case Report.Cureus · 2026Article
- Langerhans cell histiocytosis in the adult lower gastrointestinal tract.BMJ case reports · 2026Article
- ARAF mutated langerhans cell histiocytosis developed on the background of ARAF/JAK2 mutated polycythaemia vera: evidence of branching evolution from the same progenitor cell. A case report and literature review.Annals of hematology · 2026Review
- No detectable infectious agents in Langerhans cell histiocytosis with lung involvement.ERJ open research · 2026Article
- Origin, diagnosis and treatment of periocular Histiocytic disorders- state-of-the-art review.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2026Review
- A case of mixed histiocytosis (Erdheim-Chester disease and Langerhans cell histiocytosis) with STEAP3-associated anemia and type 4 hemochromatosis.Orphanet journal of rare diseases · 2026Article
- Predictive value of metabolic parameters from PET/CT in adult multifocal Langerhans cell histiocytosis.Annals of nuclear medicine · 2026Article
97 more citing papers are in PubMed but not listed here.
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Authors and funding
18 authors at 15 institutions in 7 countries.
Funding
Abstract
Langerhans cell histiocytosis (LCH) can affect children and adults with a wide variety of clinical manifestations, including unifocal, single-system multifocal, single-system pulmonary (smoking-associated), or multisystem disease. The existing paradigms in the management of LCH in adults are mostly derived from the pediatric literature. Over the last decade, the discovery of clonality and MAPK-ERK pathway mutations in most cases led to the recognition of LCH as a hematopoietic neoplasm, opening the doors for treatment with targeted therapies. These advances have necessitated an update of the existing recommendations for the diagnosis and treatment of LCH in adults. This document presents consensus recommendations that resulted from the discussions at the annual Histiocyte Society meeting in 2019, encompassing clinical features, classification, diagnostic criteria, treatment algorithm, and response assessment for adults with LCH. The recommendations favor the use of 18F-Fluorodeoxyglucose positron emission tomography-based imaging for staging and response assessment in the majority of cases. Most adults with unifocal disease may be cured by local therapies, while the first-line treatment for single-system pulmonary LCH remains smoking cessation. Among patients not amenable or unresponsive to these treatments and/or have multifocal and multisystem disease, systemic treatments are recommended. Preferred systemic treatments in adults with LCH include cladribine or cytarabine, with the emerging role of targeted (BRAF and MEK inhibitor) therapies. Despite documented responses to treatments, many patients struggle with a high symptom burden from pain, fatigue, and mood disorders that should be acknowledged and managed appropriately.
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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.