pulmonary Langerhans cell histiocytosis


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pulmonary Langerhans cell histiocytosis

A condition seen almost exclusively in cigarette smokers, which is characterised by proliferation of Langerhans cells. Early lesions have a centrilobular distribution and a mixed inflammatory-cell infiltrate consisting of lymphocytes, eosinophils, neutrophils, plasma cells, pigmented macrophages and fibroblasts. With time, the inflammation subsides and fibrotic scars predominate with enlarged and distorted airspaces. The characteristic histology can be confirmed by CD1a staining.
References in periodicals archive ?
Pulmonary Langerhans cell histiocytosis diagnosed by core needle biopsy of the lung.
Pulmonary Langerhans cell histiocytosis in an infant: Can passive smoking accelerate the disease progress?
Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans.
Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis? Am J Respir Crit Care Med.
Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004;125:1028-32.
Pulmonary Langerhans cell histiocytosis: an update from the pathologists' perspective.
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare lung disease caused by the accumulation of inflammatory cells in small airways of the lung, leading to nodular granulomatous changes of the lung parenchyma, which over time, progress to reticular and cystic changes.
Numerous less common and rare entities are also discussed, including idiopathic nonspecific interstitial pneumonia (NSIP), advanced pulmonary Langerhans cell histiocytosis (PLCH) and smokingrelated interstitial fibrosis, end-stage pulmonary sarcoidosis, Erdheim-Chester disease (ECD), and Hermansky-Pudlak syndrome.
They compared and contrasted the clinical, radiologic, and histologic characteristics of various causes of advanced fibrotic ILD, including connective tissue disease-associated ILD, chronic hypersensitivity pneumonitis, advanced pulmonary Langerhans cell histiocytosis, end-stage pulmonary sarcoidosis, Erdheim-Chester disease, and Hermansky-Pudlak syndrome.
Pulmonary Langerhans cell histiocytosis (PLCH) is the involvement of the lung by LCH.
Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy.

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