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

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/61989592:15110/16:33154988

Výsledek na webu

—

DOI - Digital Object Identifier

—

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

FD - Onkologie a hematologie

OECD FORD obor

—

Projekt

<a href="/cs/project/NT11103" target="_blank" >NT11103: Analýza molekulárních subtypů difúzního velkobuněčného lymfomu (DLBCL) metodou arrayCGH s vysokým rozlišením pro určení genových změn prognostického významu</a><br>

Návaznosti

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

Rok uplatnění

2016

Kód důvěrnosti údajů

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

Název periodika

Pathology and Oncology Research

ISSN

1219-4956

e-ISSN

—

Svazek periodika

22

Číslo periodika v rámci svazku

2

Stát vydavatele periodika

NG - Nigérijská federativní republika

Počet stran výsledku

11

Strana od-do

233-243

Kód UT WoS článku

—

EID výsledku v databázi Scopus

—