Lung-nontumor - Amyloidosis

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Lung-nontumor

Other non-neoplastic disease

Amyloidosis

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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● Categorized by clinical syndrome (systemic or localized) and biochemical type
● Currently 25 distinct types of amyloid fibers
● Four major types are AL protein (amyloid light chain), AA protein (amyloid associated), ATTR protein (transthyretin, formerly prealbumin) and B2-microglobulin

In lung, four major clinical syndromes:
● Systemic (generalized) pulmonary amyloid
● Localized pulmonary amyloid (tracheobronchial and nodular forms)
● Diffuse interstitial septal amyloidosis (diffuse parenchymal amyloid, interstitial, septal or vascular amyloid)
● Pleural amyloid (very rare)

Systemic pulmonary amyloidosis
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● Primary (nearly always AL type associated with monoclonal plasma cell proliferations), secondary/reactive (AA type) or senile (ATTR type)
● Primary amyloidosis has worse pulmonary disease than secondary type; lungs involved in 36-92% of cases, but only rarely cause significant symptomatology relative to underlying malignancy
● Secondary most often associated with rheumatoid arthritis, chronic osteomyelitis, and malignancy
● May be seen with HIV infection and lung transplant
● Increasing number of cases associated with cystic fibrosis
● Senile amyloid is usually an incidental finding in elderly patients with cardiac amyloid
● Lung disease is usually mild, involvement is similar to cardiac disease

Tracheobronchial amyloidosis

General
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● Diffuse narrowing of airway
● Symptoms of hemoptysis, atelectasis, obstruction and asthma
● May have polypoid nodules
● 15-40% die at mean 9 years after diagnosis from respiratory failure, pulmonary hemorrhage and pneumonia
● Does not evolve into systemic amyloidosis
● Diagnosis: bronchial biopsy

Case reports
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● 49 year old man (Arch Pathol Lab Med 2003;127:e420)

Treatment
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● Laser therapy or bronchoscopic removal of deposits, radiation therapy or lung transplant

Gross description
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● Focal to diffuse nodular thickening of trachea and proximal bronchial walls with patchy mural calcification
● Also extensive bronchial stenosis, post-obstructive pneumonia and atelectasis

Micro description
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● Extensive thickening of submucosa due to irregular nodular masses or sheets of amyloid; submucosal glands are reduced, chondrification, calcification or osseous metaplasia of larger airways is present
● Variable multinucleated, osteoclast-like giant cells and plasma cells within amyloid
● Also amyloid deposition within submucosal vessel walls

Gross/micro images
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Various images

Positive stains
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● Congo Red (apple-green birefringence with polarized light)

Differential diagnosis
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● Systemic amyloidosis
● Light chain deposition disease
● Pulmonary scar tissue
● Pulmonary lymphoproliferative disorders
● Tracheobronchopathia osteochondroplastica: submucosal bony and cartilaginous tissue projects into tracheobronchial lumen, no amyloid

Nodular amyloid (amyloidoma)

General
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● Rare, presents as asymptomatic nodule
● Usually in asymptomatic elderly with nodule on chest Xray, but no evidence of systemic disease
● Good prognosis; typically doesn't progress to lymphoproliferative disorders
● Nodular involvement may be associated with systemic involvement

Case reports
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● 85 year old woman with asymptomatic pulmonary nodules (Case of Week #197)

Micro description
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● Well circumscribed amyloid, often with lymphocytes (T cells) and plasma cells
● Granulomatous reaction to amyloid common, often calcification and ossification

Micro images
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Pulmonary amyloidoma

Amyloidosis (figures 3 and 4; 4 is Congo Red staining)

Positive staining
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● Congo Red staining: glassy, salmon-pink amorphous material with apple green birefringence under polarized microscopy
● Clonal restriction of plasma cells (kappa or lambda staining, not both)

EM description
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● Amyloid fibrils

EM images
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Fibrils

Differential diagnosis
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● Primary pulmonary lymphomas with amyloid production: <1% of pulmonary lymphomas have amyloid deposits, usually age 70+ years with marginal zone or SLL/CLL subtypes, often with lymphatic tracking and reactive lymphoid follicles; the presence of evenly distributed lymphocytes in the nodule and invasion of the pleura is specific for lymphoma
● Hyalinizing granuloma: history of exposure to Histoplasma or TB, collagen is Congo-red negative

Diffuse interstitial septal amyloidosis
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● Similar symptoms as diffuse interstitial fibrosis
● May be caused by systemic amyloidosis
● Diffuse interstitial deposits of amyloid involving alveolar septa; also involvement of pulmonary arteries
● Associated with systemic amyloidosis, congestive heart failure and pulmonary edema
● No effective treatment; poor prognosis

End of Lung-nontumor > Other non-neoplastic disease > Amyloidosis

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