Primary amyloidosis pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Sabawoon Mirwais, M.B.B.S, M.D.[3]

Overview

Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes. In primary amyloidosis, amyloid gradually accumulate and amyloid deposition is widespread in the viscera (mainly kidneys, heart and liver), blood vessel walls, nerves and in different connective tissues. Primary (AL) amyloidosis) is the most common type of amyloidosis. It results from aggregation and deposition of monoclonal immunoglobulin (Ig) light chains that usually produced by plasma cell clones.

Pathophysiology

Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes. In primary amyloidosis, amyloid gradually accumulate and amyloid deposition is widespread in the viscera (mainly kidneys, heart and liver), blood vessel walls, and in different connective tissues.

Pathogenesis

Amyloids

Light chain amyloid or AL amyloid

Role of plasma cells in primary (AL) amyloidosis

Interaction of amyloid fibrils with microenvironment

Tissue damage

Associated Conditions

Primary amyloidosis has been associated with the following conditions:

Gross Pathology

Nodular depostis of amyloid on pleural surface, courtesy: Wikimedia commons


Microscopic Pathology

Small bowel duodenum with amyloid deposition Congo red.[4]
Amyloidosis (black arrows) in a lymph node after staining with Congo Red.[5]
Green birefringence under polarized light.[6]
Hepatic amyloidosis, Courtesy:wikimedia commons


References

  1. Riek, Roland; Eisenberg, David S. (2016). "The activities of amyloids from a structural perspective". Nature. 539 (7628): 227–235. doi:10.1038/nature20416. ISSN 0028-0836.
  2. Perfetti V, Palladini G, Casarini S, Navazza V, Rognoni P, Obici L, Invernizzi R, Perlini S, Klersy C, Merlini G (January 2012). "The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44". Blood. 119 (1): 144–50. doi:10.1182/blood-2011-05-355784. PMID 22067386.
  3. Lavatelli F, Imperlini E, Orrù S, Rognoni P, Sarnataro D, Palladini G, Malpasso G, Soriano ME, Di Fonzo A, Valentini V, Gnecchi M, Perlini S, Salvatore F, Merlini G (November 2015). "Novel mitochondrial protein interactors of immunoglobulin light chains causing heart amyloidosis". FASEB J. 29 (11): 4614–28. doi:10.1096/fj.15-272179. PMID 26220173.
  4. By Michael Feldman, MD, PhDUniversity of Pennsylvania School of Medicine - http://www.healcentral.org/healapp/showMetadata?metadataId=38717, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=870218
  5. By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377559787/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629716
  6. By Ed Uthman, MD - https://www.flickr.com/photos/euthman/377559955/, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=1629705