ArticleBlood advances2023
Long-term outcomes among adults with Langerhans cell histiocytosis.
Article in Blood advances, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 21 citations in OpenAlex.
- Phase 2 study using low dose cytarabine for adult patients with newly diagnosed Langerhans cell histiocytosis.Leukemia · 2024Trial
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- Langerhans cell histiocytosis in the adult lower gastrointestinal tract.BMJ case reports · 2026Article
- Frequent NPM1 mutation, monoblastic/monocytic origin and prognostic significance of organ and system involvement in myeloid sarcoma: a multicenter study.The journal of pathology. Clinical research · 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
- Histiocyte Society blueprint for Langerhans cell histiocytosis research: from cell-of-origin to a more comprehensive cure.Haematologica · 2025Review
- Impact of Sex on Lung Function in Adult Langerhans Cell Histiocytosis.Life (Basel, Switzerland) · 2025Article
- Approach to the Patient: From Endocrinopathy to the Diagnosis of a Histiocytic Disorder.The Journal of clinical endocrinology and metabolism · 2025Review
- [European journal of nuclear medicine and molecular imaging · 2025Article
- Spontaneous Regression of Adult Multi-system Langerhans Cell Histiocytosis Presenting as Liver Tumor Rupture.Internal medicine (Tokyo, Japan) · 2025Review
- Article
- A Case of Langerhans Cell Histiocytosis With a Neck Mass as the First Symptom.Clinical case reports · 2025Article
- Langerhans Cell Histiocytosis and Other Histiocytic Lesions.Head and neck pathology · 2025Review
- Liver Transplantation for Isolated Langerhans Cell Histiocytosis in an Adult.Case reports in hematology · 2025Article
- Pulmonary Langerhans cell histiocytosis: A case series and literature review.Journal of clinical imaging science · 2025Article
- Adult onset multisystem Langerhans cell histiocytosis initially presenting with arginine vasopressin deficiency: a case report.Frontiers in medicine · 2025Article
- Langerhans cell histiocytosis: NACHO update on progress, chaos, and opportunity on the path to rational cures.Cancer · 2024Review
- Multisystemic recurrent Langerhans cell histiocytosis misdiagnosed with chronic inflammation at the first diagnosis: A case report.World journal of radiology · 2024Article
- Isolated Langerhans cell histiocytosis in the stomach of adults: four-case series and literature review.Journal of hematopathology · 2024Review
- Clinical characteristics, molecular aberrations, treatments, and outcomes of malignant histiocytosis.American journal of hematology · 2024Article
Corrections and comments
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Authors and funding
20 authors at 4 institutions in 1 country.
Funding
Abstract
Advances in the treatment of Langerhans cell histiocytosis (LCH) have resulted in a growing survivor population. There is a lack of data on long-term outcomes among adults with LCH. We conducted a retrospective record review of 219 adults (aged ≥18 years) with LCH. Most common presentation was multisystem (34.2%), followed by single-system pulmonary (32%), unifocal (28.3%), and single-system multifocal (5.5%) LCH. Risk organ involvement (the liver, spleen, or bone marrow) was seen in 8.7% of cases, and 40 of 88 (45.5%) tested cases were BRAFV600E. At a median follow-up of 74 months, 5-year progression-free survival (PFS) was 58.3% and estimated median PFS was 83 months. Median overall survival (OS) was not reached; 5- and 10-year OS rates were 88.7% and 74.5%, respectively. Risk organ involvement was associated with worse PFS (hazard ratio [HR], 4.5) and OS (HR, 10.8). BRAFV600E was not associated with risk organ involvement or survival. When compared with matched unaffected US population, individuals with LCH had a significantly higher risk of overall mortality (standardized mortality ratio [SMR], 2.66), specifically among those aged <55 years at diagnosis (SMR, 5.94) and those with multisystem disease (SMR, 4.12). Second cancers occurred in 16.4% cases, including diverse hematologic and solid organ malignancies. LCH-associated deaths constituted 36.1% of deaths and occurred within 5 years of diagnosis. After 5 years, non-LCH causes of death, including second cancers, chronic obstructive pulmonary disease, and cardiovascular diseases, predominated. Our study highlights, to our knowledge, for the first time, that adults with LCH experience early and late mortality from non-LCH causes and the need for development of targeted survivorship programs to improve outcomes.
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Registered trials
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.