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Lung-nontumor

Other non-neoplastic disease

Alveolar proteinosis


Reviewers: Dr. Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 9 November 2011, last major update October 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
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● Rare disease with accumulation of acellular surfactant presumedly due to impaired clearance, likely due to dysfunction of alveolar macrophages
● Considered a response to alveolar injury, not a specific entity
● Congenital (2% of cases), primary (idiopathic) or secondary forms
● Congenital cases are usually due to mutation in surfactant protein or GM-CSF receptor genes
● Secondary (most common), associated with silica dust, aluminum, fiberglass, chemicals, immunosuppression, leukemia / lymphoma, Nocardia, Mycobacteria and Aspergillosis
● Usually adults (more common in smokers), also children
● Sputum contains chunks of gelatinous material; no inflammation
● Usually does NOT progress to chronic fibrosis
Xray: resembles pulmonary edema; diffuse pulmonary opacification

Treatment
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● Whole lung lavage (also diagnostic)

Micro description
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● Alveoli contain amorphous, eosinophilic and PAS+ material in lumina consisting of type II pneumocytes, lamellar bodies and necrotic alveolar macrophages
● Variable fibrosis
● Mild/no lymphocytic infiltration

Virtual slides
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Alveolar proteinosis

EM description
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● Exudate contains lamellar bodies, lipid particles and cellular debris

End of Lung-nontumor > Other non-neoplastic disease > Alveolar proteinosis


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