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Ups J Med Sci. 2012 May;117(2):244-50. doi: 10.3109/03009734.2012.654861. Epub 2012 Feb 15.

Localized AL amyloidosis: a suicidal neoplasm?

Author information

1
Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden. Per.Westermark@igp.uu.se

Abstract

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.

PMID:
22335280
PMCID:
PMC3339556
DOI:
10.3109/03009734.2012.654861
[Indexed for MEDLINE]
Free PMC Article

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