Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13; q32) Journal Article


Authors: Iida, S.; Pulivarthi H, R.; Ueda, R.; Chaganti, R. S. K.; Dalla-Favera, R.
Article Title: Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13; q32)
Abstract: B-cell non-Hodgkin's lymphoma (NHL) consists of heterogeneous subtypes based on histologic, immunophenotypic, and clinical findings. Recent advances in molecular biology have provided us new insights into the pathogenesis of this neoplasm at the genetic level, such as the deregulation of the protooncogenes adjoining the immunoglobulin gene (Ig) loci, which is a specific event in mature B-cell tumors. Moreover, involvement of certain protooncogenes corresponds to certain subtypes of NHL. Recently, we found that t(9;14)(p13;q32) chromosomal translocation associated with lymphoplasmacytic lymphoma (LPL) juxtaposes PAX-5 gene encoding for an essential transcription factor (BSAP: B-cell specific activator protein) for B-cell proliferation and differentiation to the Ig heavy chain gene (IgH) locus. This results in deregulated expression of the PAX-5 mRNA. We also developed a diagnostic FISH (fluorescence in situ hybridization) procedure which is able to detect 80% of the widely scattering 9p13 breakpoints involved in this translocation. Thus, an understanding of the PAX-5 gene's physiological role in B-cell development and the pathological role in tumorigenesis may lead to the optimal clinical treatment strategy for LPL and LPL-derived diffuse large cell lymphoma (DLCL).
Keywords: dna-binding proteins; clinical feature; pathogenesis; review; lymphocyte proliferation; animals; proto oncogene; chromosome 9p; gene locus; chromosomes, human, pair 9; transcription factor; cell differentiation; histology; b lymphocyte; carcinogenesis; transcription factors; nuclear proteins; b cell lymphoma; lymphoma, b-cell; nonhodgkin lymphoma; fluorescence in situ hybridization; immunoglobulin heavy chain; gene rearrangement; immunoglobulin gene; messenger rna; lymphoma; chromosome rearrangement; chromosome translocation; translocation, genetic; immunophenotyping; large cell lymphoma; molecular biology; chromosome 14q; deregulation; chromosomal translocation; messenger rna synthesis; chromosomes, human, pair 14; b-cell-specific activator protein; bsap; humans; human; priority journal; t(9; q32); lymphoplasmacytic lymphoma (lpl); pax-5; 14) (p13; lymphoma, small lymphocytic
Journal Title: Leukemia and Lymphoma
Volume: 34
Issue: 1-2
ISSN: 1042-8194
Publisher: Taylor & Francis Group  
Date Published: 1999-01-01
Start Page: 25
End Page: 33
Language: English
PUBMED: 10350329
PROVIDER: scopus
DOI: 10.3109/10428199909083377
DOI/URL:
Notes: Review -- Export Date: 16 August 2016 -- Source: Scopus
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MSK Authors
  1. Raju S K Chaganti
    260 Chaganti