Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Amyloidosis, Pleural

  • Michelle A. FoshatEmail author
Reference work entry


Pleural type; Pulmonary amyloidosis


Pleural amyloidosis is the deposition of amyloid protein into the visceral or parietal pleura. Amyloid refers to misfolded proteins with a beta-pleated sheet conformation forming insoluble fibrils that accumulate in various organs. Amyloid buildup mechanically disrupts the involved tissue parenchyma and interferes with many cellular processes occurring within the cellular microenvironment. The extent of pathophysiological disturbances due of amyloid is still incompletely understood.

Over 20 types of proteins accompanying different diseases can result in amyloid formation. The most commonly involved misfolded fibril proteins are AL, AA, ATTR, and Aβ amyloid. AL amyloid is derived from excess light-chain immunoglobulins associated with plasma cells dyscrasias: most notably multiple myeloma. The precursor protein of AA amyloid is serum amyloid A (SAA) proteins, a subset of apolipoproteins, which are acute phase reactants elevated in...

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References and Further Reading

  1. 1.
    Berk, J. L., Keane, J., Seldin, D. C., Sanchorawala, V., Koyama, J., Dember, L. M., & Falk, R. H. (2003). Persistent pleural effusions in primary systemic amyloidosis. Chest, 124(3), 969–977.CrossRefPubMedGoogle Scholar
  2. 2.
    Gertz, M. A., Buadi, F. K., Zeldenrush, S. R., & Hayman, S. R. (2013). Chapter 87: Immunoglobulin light-chain amyloidosis (Primary amyloidosis). In R. Hoffman, E. J. Benz Jr., L. E. Silberstein, E. H. Helen, & J. I. Weitz (Eds.), Hematology: Basic principles and practice (6th ed., pp. 1350–1373). Philadelphia, PA: Saunders, and imprint of Elsevier.Google Scholar
  3. 3.
    Gillmore, J. D., & Hawkins, P. N. (2013). Pathophysiology and treatment of systemic amyloidosis. Nature Reviews Nephrology, 9(10), 574–586.CrossRefPubMedGoogle Scholar
  4. 4.
    Merlini, G., & Bellotti, V. (2003). Molecular mechanisms of amyloidosis. New England Journal of Medicine, 349, 583–596.CrossRefPubMedGoogle Scholar
  5. 5.
    Rafii, R., Leslie, K., Heo, J., & Chan, A. (2011). A 71-year-old woman with an unusual cause for pleural effusions. Chest, 139(5), 1237–1241.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PathologyUniversity of Texas Medical Branch at GalvestonGalvestonUSA