Other non-neoplastic disease
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.
● 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
● Whole lung lavage (also diagnostic)
● 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
● Exudate contains lamellar bodies, lipid particles and cellular debris
End of Lung-nontumor > Other non-neoplastic disease > Alveolar proteinosis
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