Publication Cover
Amyloid
The Journal of Protein Folding Disorders
Volume 13, 2006 - Issue 3
619
Views
141
CrossRef citations to date
0
Altmetric
Original

The spectrum of localized amyloidosis: A case series of 20 patients and review of the literature

, &
Pages 135-142 | Published online: 06 Jul 2009
 

Abstract

Localized deposition of amyloid may occur in individual organs, in the absence of systemic involvement. The reason for localized deposition is unknown, but it is hypothesized that deposits result from local synthesis of amyloid protein, rather than the deposition of light chains produced elsewhere. We identified 20 cases of localized amyloidosis at our institution between 1993 and 2003. There were 11 males and nine females in the group. The mean age at the time of diagnosis was 65.5 years. Organs involved included skin, soft tissues, oropharynx, larynx, lung, bladder, colon, conjunctiva, and lymph node. In six of nine patients typed, the amyloid light chain was lambda. In those patients where follow-up was available (mean 7.6 years), none developed systemic disease. Localized amyloidosis occurs in a variety of organ systems. Evolution into systemic amyloidosis was not seen in our series of patients, supporting the hypothesis of local production of amyloid protein in these cases.

Abbreviations
AL=

immunoglobulin light chain

2M=

β2-microglobulin

ATTR=

transthyretin

AA=

(Apo)serumAA

DNA=

deoxyribonucleic acid

Abbreviations
AL=

immunoglobulin light chain

2M=

β2-microglobulin

ATTR=

transthyretin

AA=

(Apo)serumAA

DNA=

deoxyribonucleic acid

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.