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Prepublished online as a Blood First Edition Paper on December 19, 2002; DOI 10.1182/blood-2002-09-2707. This Article Full Text (PDF) All Versions of this Article: 2002-09-2707v1 101/10/3801    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Abraham, R. S Articles by Fonseca, R. Search for Related Content PubMed PubMed Citation Articles by Abraham, R. S Articles by Fonseca, R. Social Bookmarking                   What's this? Submitted September 4, 2002 Accepted December 5, 2002 Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated (AL) amyloidosis Roshini S Abraham, Susan M Geyer, Tammy L Price-Troska, Cristine Allmer, Robert A Kyle, Morie A Gertz, and Rafael Fonseca* Division of Clinical Biochemistry and Immunology, Mayo Clinic Rochester, Rochester, MN, USA Division of Hematology, Mayo Clinic Rochester, Rochester, MN, USA Division of Biostatistics, Mayo Cancer Center, Mayo Clinic Rochester, Rochester, MN, USA * Corresponding author; email: fonseca.rafael{at}mayo.edu. Light chain (AL) amyloidosis is a plasma cell dyscrasia in which the secreted monoclonal immunoglobulin (Ig) light chains form amyloid fibrils. There is considerable heterogeneity in clinical presentation, and prognosis of the disease relates to the severity of organ dysfunction induced by amyloid deposits. The mechanisms by which the amyloid fibrils are deposited as well as the predilection for specific organ sites have not been clearly elucidated. This study characterizes the repertoire of immunoglobulin light chain variable genes used by the clonal B cell in AL amyloid patients, and the association of light chain variable region (VL) genes with clinical presentation and outcome is assessed in 58 (32 and 26 ) patients. A preferential use of VL germline genes was noted for both AL and patients. There was a significant correlation between the use of the VVI germline donor, 6a and renal involvement as well as the V III gene, 3r with 'soft-tissue' AL. The use of a biased VL gene repertoire also correlated with clinical outcome, revealing important trends for predicting prognosis. The use of V II germline genes was associated with cardiac amyloidosis and affected survival adversely. The presence of multiple myeloma also correlated with a poor prognosis. The presence of renal disease, on the other hand, was associated with improved survival. Therefore, identification of the clonal VL gene in AL has important implications in determining clinical outcome. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. A. Vrana, J. D. Gamez, B. J. Madden, J. D. Theis, H. R. Bergen III, and A. Dogan Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens Blood, December 3, 2009; 114(24): 4957 - 4959. [Abstract] [Full Text] [PDF] B. K. Arendt, M. Ramirez-Alvarado, L. A. Sikkink, J. J. Keats, G. J. Ahmann, A. Dispenzieri, R. Fonseca, R. P. Ketterling, R. A. Knudson, E. M. Mulvihill, et al. Biologic and genetic characterization of the novel amyloidogenic lambda light chain-secreting human cell lines, ALMC-1 and ALMC-2 Blood, September 1, 2008; 112(5): 1931 - 1941. [Abstract] [Full Text] [PDF] E. M. 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