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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 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 Citation Map 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. Related Collections Immunobiology Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 May 2003, Vol. 101, No. 10, pp. 3801-3807 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL) Roshini S. Abraham, Susan M. Geyer, Tammy L. Price-Troska, Cristine Allmer, Robert A. Kyle, Morie A. Gertz, and Rafael Fonseca From the Division of Clinical Biochemistry and Immunology, the Division of Hematology, and the Division of Biostatistics, Mayo Cancer Center, Mayo Clinic, Rochester, MN. Light chain-associated amyloidosis (AL) 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 germ-line genes was noted for both AL  and  patients. There was a significant correlation between the use of the V VI germ-line 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 germ-line 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. © 2003 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: 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. Baden, B. A. L. Owen, F. C. Peterson, B. F. Volkman, M. 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