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Pulmonary Langerhans’ cell histiocytosis 

Pulmonary Langerhans’ cell histiocytosis
Pulmonary Langerhans’ cell histiocytosis

S.J. Bourke

and D.J. Hendrick


July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 24 September 2021

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....

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