A novel non-immunoglobulin (non-Ig)/BCL6 translocation in diffuse large B-cell lymphoma involving chromosome 10q11.21 loci and review on clinical consequences of BCL6 rearrangements

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00098892%3A_____%2F16%3AN0000138" target="_blank" >RIV/00098892:_____/16:N0000138 - isvavai.cz</a>

Alternative codes found

RIV/61989592:15110/16:33154988

Result on the web

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DOI - Digital Object Identifier

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Result language

angličtina

Original language name

A novel non-immunoglobulin (non-Ig)/BCL6 translocation in diffuse large B-cell lymphoma involving chromosome 10q11.21 loci and review on clinical consequences of BCL6 rearrangements

Original language description

BCL6 rearrangements (3q27) are the most common chromosomal abnormalities in diffuse large B-cell lymphoma (DLBCL), with numerous immunoglobulin (Ig) and non-Ig genes as partners. In DLBCL, the translocations occur predominantly in the “major breakpoint region” encompassing the first noncoding exon and a part of the first intron of BCL6; few cases with “alternative breakpoint cluster” located 245–285 kb 5′ BCL6 were also described. The regulatory sequences of known Ig and non-Ig partners replace the 5′ untranslated region of the BCL6 in the same transcriptional orientation. Contrary to Ig/BCL6 fusions typical by high BCL6 gene expression, in non-Ig/BCL6 translocations were observed unexpectedly low BCL6 mRNA levels. From the clinical point of view, the survival rate of DLBCL patients with non-Ig partners is inferior to those with Ig/BCL6 translocations, suggesting that non-Ig/BCL6 fusion is a poor prognostic indicator. Hereby we provide comprehensive information about known non-Ig translocation partners and clinical consequences of BCL6 rearrangements in DLBCL. Moreover, we describe a novel reciprocal translocation t(3;10) in refractory patient with DLBCL with the breaking points at 5′ untranslated region of BCL6 and 5′ untranslated region of the RASGEF1A gene on chromosome 10q11.21 loci; this rearrangement was associated with low BCL6 and RASGEF1A gene expressions. Our patient harbouring dual chromosomal rearrangement involving BCL2 and BCL6 genes relapsed three-times and died soon; thus, further supporting the notion that non-Ig/BCL6 fusion is a poor prognostic indicator of DLBCL. There is evidence of prognostic value of BCL6 rearrangements also in rituximab era.

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FD - Oncology and haematology

OECD FORD branch

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Project

<a href="/en/project/NT11103" target="_blank" >NT11103: The analysis of molecular subtypes of diffuse large B-cell lymphoma by high-resolution arrayCGH for determination of genetic changes of prognostic importance</a><br>

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year

2016

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Pathology and Oncology Research

ISSN

1219-4956

e-ISSN

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Volume of the periodical

22

Issue of the periodical within the volume

2

Country of publishing house

NG - NIGERIA

Number of pages

11

Pages from-to

233-243

UT code for WoS article

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EID of the result in the Scopus database

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