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Pulmonary Langerhans’ cell histiocytosis 

Pulmonary Langerhans’ cell histiocytosis
Chapter:
Pulmonary Langerhans’ cell histiocytosis
Author(s):

S. J. Bourke

DOI:
10.1093/med/9780198746690.003.0425
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date: 28 February 2021

Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles, resulting in inflammatory nodules, cyst formation, and fibrosis. Langerhans’ cells are a particular type of histiocyte derived from dendritic cells in the bone marrow. They normally migrate in the blood to the squamous epithelium of the skin, lungs, gastrointestinal, and female genital tract, where they are involved in antigen presentation to T cells. It presents with cough, breathlessness, and (sometimes) systemic symptoms. Chest radiography and CT typically show nodules which then cavitate and may rupture, causing pneumothorax. Corticosteroids and/or cytotoxic drugs are of some benefit, and lung transplantation is an option for progressive disease.

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