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BCL3 rearrangement and t(14;19)(q32;q13) in lymphoproliferative disorders

✍ Scribed by Lucienne Michaux; Cristina Mecucci; Michel Stul; Iwona Wlodarska; Jesus Maria Hernandez; Peter Meeus; Jean-Louis Michaux; Jean-Marie Scheiff; Henri Noël; Andries Lodwagie; Arnold Criel; Marc Boogaerts; Angeline Van Orshoven; Jean-Jacques Cassiman; Herman Van den Berghe

Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
695 KB
Volume
15
Category
Article
ISSN
1045-2257

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✦ Synopsis

Translocation t( 14; I9)(q32;q 13) is a rare but recurrent abnormatiy in chronic lymphocytic leukemia and small cell lymphoma.

It has been associated with rearrangements of the BCU gene, which is located at the breakpoint on chromosome 19 and is juxtaposed to the immunoglobutin heavy chain tocus on chromosome 14 as a result of the translocation. This results in transcriptional up-regulation of the BCU gene, which encodes a transcription coactivator, an I-KB protein, probably contributing to disease progression. We found, among 4,487 cytogenetic analyses of Iymphoproliferative disorders. six cases with a t( 14; I9)(q32;q 19, five of which showed the classical t( 14; 19)(q32;q 13) and one of which showed a three-way translocation t(7; 19; I4)(q2 I ;q I 3;q32). The 14; I9 translocation never occurred as a single abnormatitr; additional aberrations included trisomy I 2 and several structural abnormalities. The cytogenetic examination was supplemented by molecular analysis using available probes for the BCU locus (pat .4P and pa5B) in I, t 50 of the 4,487 patients. Rearrangements of Kl3 could be detected in five cases, all of which had the classical t( L4; 19). In the case with t(7; 19; 14). the suspected BCU involvement could only be confirmed using long-range restriction mapping, indicating that, with the usually available BCU probes, rearrangements of this locus may be missed. Genes Chromosom Concer 15t38-47 (1996). 0

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