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Localized AL amyloidosis: A suicidal neoplasm?
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Molecular and Morphological Pathology. (Per Westermark)
2012 (English)In: Upsala Journal of Medical Sciences, ISSN 0300-9734, E-ISSN 2000-1967, Vol. 117, no 2, 244-250 p.Article, review/survey (Refereed) Published
Abstract [en]

Although AL amyloidosis usually is a systemic disease, strictly localized AL deposits are not exceptionally rare. Such case reports form a considerable body of published articles. Although both AL amyloidosis types are formed from an N-terminal segment of a monoclonal immunoglobulin light chain, a typical localized AL amyloid differs from the systemic counterpart by the morphological appearance of the amyloid, and presence of clonal plasma cells and of giant cells. In this article it is pointed out that localized AL amyloidosis ('amyloidoma') represents a true plasma cell neoplasm and not a pseudotumor. The pathogenesis of localized AL amyloidosis may differ from that of the systemic type, a suggestion underlined by the fact that localized AL amyloidosis of kappa type is as common as that of lambda origin, in contrast to the systemic form where lambda chains constitute the overwhelming majority of cases. It is suggested that oligomeric assemblies of the produced immunoglobulin light chain are toxic to plasma cells, which in this way commit suicide.

Place, publisher, year, edition, pages
2012. Vol. 117, no 2, 244-250 p.
Keyword [en]
Amyloid, immunoglobulin light chain, fibrils, giant cells, plasmacytoma, neoplasm
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-174661DOI: 10.3109/03009734.2012.654861ISI: 000302949200018OAI: oai:DiVA.org:uu-174661DiVA: diva2:528454
Available from: 2012-05-25 Created: 2012-05-24 Last updated: 2012-05-25Bibliographically approved

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