Chapter

Pulmonary Langerhans’ cell histiocytosis

S.J. Bourke and D.J. Hendrick

in Oxford Textbook of Medicine

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online May 2010 | e-ISBN: 9780199570973 | DOI: http://dx.doi.org/10.1093/med/9780199204854.003.181405

Series: Oxford Textbooks

Pulmonary Langerhans’ cell histiocytosis

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Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles. It presents, nearly always in smokers, with cough, breathlessness, and (sometimes) systemic symptoms. Pneumothorax (sometimes bilateral) is a common complication. Chest radiography typically shows micronodules, reticulation, and small cysts, with fibrosis in advanced cases. Patients must stop smoking. About 25% of cases resolve, 50% stabilize, and 25% lose lung function. Corticosteroids and/or cytotoxic drugs are usually given for progressive disease, but the benefits are unclear....

Chapter.  788 words.  Illustrated.

Subjects: Respiratory Medicine and Pulmonology

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