Clinical Outcomes of Pulmonary Langerhans'-Cell Histiocytosis in Adults
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Open Access
- 14 February 2002
- journal article
- research article
- Published by Massachusetts Medical Society in The New England Journal of Medicine
- Vol. 346 (7), 484-490
- https://doi.org/10.1056/nejmoa012087
Abstract
Pulmonary Langerhans'-cell histiocytosis is an uncommon interstitial lung disease in adults. It has an unpredictable course and may be associated with an increased susceptibility to the development of malignant neoplasms. We reviewed the records of 102 adults with histopathologically confirmed pulmonary Langerhans'-cell histiocytosis to ascertain their vital status and whether cancer had been diagnosed. The health status of surviving patients was quantified with the use of the 36-Item Short-Form General Health Survey. Factors potentially associated with survival after the diagnosis of pulmonary Langerhans'-cell histiocytosis were analyzed with the Cox proportional-hazards model. The median follow-up period was 4 years (range, 0 to 23). There were 33 deaths, 15 of which were attributable to respiratory failure. Six hematologic cancers were diagnosed. The overall median survival was 12.5 years, which was significantly shorter than that expected for persons of the same sex and calendar year of birth (P1) (P=0.004), a higher residual volume (P=0.007), a lower ratio of FEV1 to forced vital capacity (P=0.03), and a reduced carbon monoxide diffusing capacity (P=0.001). The survival of adults with pulmonary Langerhans'-cell histiocytosis is shorter than that in the general population, and respiratory failure accounts for a substantial proportion of deaths among such patients.Keywords
This publication has 26 references indexed in Scilit:
- Pulmonary Langerhans'-Cell HistiocytosisThe New England Journal of Medicine, 2000
- Langerhans cell histiocytosis in the adultMedical and Pediatric Oncology, 1996
- Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Societe de Pathologie Thoracique du NordEuropean Respiratory Journal, 1996
- Acute leukemia in association with langerhans cell histiocytosisMedical and Pediatric Oncology, 1994
- Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X) A Clinicopathologic Study of 48 CasesThe American Journal of Surgical Pathology, 1993
- Langerhans’ Cell Histiocytosis and Malignancies of the M-PIRE SystemAmerican Journal of Clinical Pathology, 1993
- Association of langerhans cell histiocytosis with malignant neoplasmsCancer, 1993
- Histiocytosis X in the lungHuman Pathology, 1983
- Langerhansʼ cell granulomatosis (histiocytosis X) associated with malignant lymphomasThe American Journal of Surgical Pathology, 1983
- Eosinophilic Granuloma of LungMedicine, 1981