ArticleBMC cancer2020
Clinical presentation and prognostic analysis of adult patients with Langerhans cell histiocytosis with pulmonary involvement.
Article in BMC cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 19 citations in OpenAlex.
- Vitamin D levels and skeletal quality of life in adult Langerhans cell histiocytosis.Endocrine connections · 2026Article
- Pulmonary langerhans cell histiocytosis following resection of lung adenocarcinoma: a case report and diagnostic challenges.American journal of cancer research · 2026Article
- Mixed Erdheim-Chester disease with thoraco-abdominal involvement.Acta radiologica open · 2025Article
- Impact of Sex on Lung Function in Adult Langerhans Cell Histiocytosis.Life (Basel, Switzerland) · 2025Article
- High resolution chest computed tomography responses for a cohort of adult with pulmonary Langerhans cell histiocytosis.Annals of hematology · 2025Article
- Circulating monocytes decrease significantly following disease-directed therapy and may reflect disease expansion in Langerhans Cell Histiocytosis.Annals of hematology · 2024Article
- Langerhans cell histiocytosis in adolescent patients: a single-centre retrospective study.Orphanet journal of rare diseases · 2022Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
backgroundThe study aimed to investigate the clinical features and prognosis factors of adult patients with Langerhans cell histiocytosis (LCH) with pulmonary involvement, especially multisystem (MS) LCH with pulmonary involvement.
methodsWe retrospectively analyzed the demographic materials, clinical features and treatment outcomes of 119 adult LCH patients with pulmonary involvement at our center from January 1990 to November 2019.
resultsAmong 119 patients, 13 (10.9%) had single-system (SS) LCH, and 106 (89.1%) had MS-LCH with pulmonary involvement. SS-LCH patients had higher smoking rate (84.6% vs 52.8%, P = 0.026) and smoking index (300 vs 200, P = 0.019) than MS-LCH patients. The percentage of respiratory symptoms of SS-LCH patients was higher than MS-LCH patients (84.6% vs 53.8%, P = 0.034). Pulmonary function was impaired in 83.8% of the patients, and DLCO was the parameter most frequently impaired, accounting for 81.1%. The median DLCO was 65.1% predicted. Patients with pneumothorax had significantly worse DLCO (P = 0.022), FEV1 (P = 0.000) and FEV1/FVC (P = 0.000) than those without pneumothorax. During the follow-up, 72.4% of the patients had stable pulmonary function, and 13.8% showed improvements after chemotherapy. The estimated 3-year OS and EFS were 89.7 and 58.3%, respectively. Patients with a baseline FEV1 ≤ 55% predicted had worse OS. A history of pneumothorax indicated worse EFS and cytarabine based therapy predicted better EFS.
conclusionsAn FEV1 ≤ 55% predicted and a history of pneumothorax at diagnosis indicated a poor prognosis. Cytarabine based regimen may arrest the decline in pulmonary function in LCH patients with pulmonary involvement and improve EFS.
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