Frequent association of t(3;14) or variant with other lymphoma‐specific translocations

Horsman, Douglas E.; McNeil, Bryan; Anderson, Michelle E.; Shenkier, Tamara; Gascoyne, Randy D.

doi:10.1111/j.1365-2141.1995.tb08365.x

Cited by 27 publications

(21 citation statements)

References 23 publications

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“…However, 3q27/BCL6 translocation sometimes coexists with other translocations in a single clone 55,56 , including t (8 ; 14)(q24 ; q32) and t (14 ; 18)(q32 ; q21) involving c-MYC and BCL2, respectively, making the role of the 3q27/BCL6 translocation in the pathogenesis of B-NHL unclear.…”

Section: Rearrangements Of Bcl6 Concurrent With Those Of C-myc And/ormentioning

confidence: 99%

Pathogenetic and Clinical Implications of Non-Immunoglobulin; BCL6 Translocations in B-Cell Non-Hodgkin's Lymphoma

Ohno

2006

J Clin Exp Hematopathol

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Chromosomal translocations affecting band 3q27, where BCL6 gene is located, are among the most common genetic abnormalities in non-Hodgkin's lymphoma of B-cell type (B-NHL). The BCL6 gene encodes a BTB/ POZ zinc finger transcription factor, which exerts repressive activity by recruiting corepressor molecules. The 3q27/BCL6 translocation is unique in that it can involve not only immunoglobulin (Ig) genes but also non-Ig chromosomal loci as a partner. To date, around 20 non-Ig partner genes have been identified. As a result of non-Ig ; BCL6 translocations, many types of regulatory sequences of each partner gene substitute for the 5′ untranslated region of BCL6, and the rearranged BCL6 comes under the control of the replaced promoter. The introduction of non-Ig ; BCL6 constructs into transformed cells led to high-level Bcl-6 protein expression in the nucleus, while BCL6 mRNA levels in clinical materials of diffuse large B-cell lymphoma (DLBCL) with non-Ig ; BCL6 translocations were unexpectedly low. A comparative study suggested that non-Ig ; BCL6 translocation and a low level of BCL6 mRNA expression are concordant indicators of a poor clinical outcome in cases of DLBCL. The coexistence of a non-Ig ; BCL6 translocation with t (14 ; 18)(q32 ; q21) in a single clone did not significantly affect the clinical features of follicular lymphoma. The pathogenetic and clinical implications of non-Ig ; BCL6 translocations in B-NHL subtypes may not be identical to those of Ig ; BCL6. 〔J Clin Exp Hematopathol 46(2) : 43-53, 2006〕

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Section: Rearrangements Of Bcl6 Concurrent With Those Of C-myc And/ormentioning

confidence: 99%

Pathogenetic and Clinical Implications of Non-Immunoglobulin; BCL6 Translocations in B-Cell Non-Hodgkin's Lymphoma

Ohno

2006

J Clin Exp Hematopathol

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“…A 3q27 translocation affecting the BCL6 gene is observed in 20-40% of DLBCL, in 15% of FL and also in a wide spectrum of B-cell lymphomas. [6][7][8] Rearrangements cluster in a 3.3 kb region termed major translocation cluster (MTC), which spans the first noncoding exon and the 5 0 region of the first intron of the gene. 6 Translocations do not only involve Ig genes, but other chromosomal loci as well, of which to date more than 20 non-Ig partners have been identified.…”

Section: Introductionmentioning

confidence: 99%

Clinical and biological relevance of single-nucleotide polymorphisms and acquired somatic mutations of the BCL6 first intron in follicular lymphoma

et al. 2005

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Genetic modifications of the BCL6 gene in lymphoma include translocations, deletions, and somatic mutations (SM) of the 5 0 noncoding region. Three single-nucleotide polymorphisms (SNPs) of the major mutation cluster region (MMC) have been reported, including two substitutions (397G/C, 502G/A) and one deletion (520DT). Clinical and biological relevance of these SNPs are unknown. Based on a case-control study, BCL6 SNPs frequencies were assessed in 97 t(14;18) follicular lymphomas (FL) and in 54 lymphomas with 3q27 rearrangement. Allele frequencies were similar in the FL and controls groups. The 397 G/C genotype was correlated to a higher-grade transformation risk (P ¼ 0.02). SM were observed in 39.1% of FL and were characterized by a clustering distribution (hot spots spanning position 420-435, 106-127, and 590-600). No correlation between genotypes or acquired mutational status and BCL6 expression was demonstrated. However, gel mobility-shift assays, using SNPs containing probes show results representative for protein/DNA complexes. This study demonstrates that the first BCL6 intron is a highly variable region as a consequence of both SNP and SM, which may contribute to biology and outcome of FL.

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“…It is also interesting to note that two cases with BCL6 gene rearrangement reported by Horsman et al (1995) were composite lymphomas with a monocytoid B-cell component.…”

Section: Discussionmentioning

confidence: 99%

BCL6 gene rearrangements also occur in marginal zone B‐cell lymphoma

Dierlamm

Pittaluga²,

Stul

et al. 1997

Br J Haematol

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Summary.Marginal zone B-cell lymphoma (MZBCL) represents a distinct subtype of B-cell non-Hodgkin's lymphoma (NHL) which has been recently recognized and defined as a disease entity. Cytogenetically, these lymphomas reveal a high prevalence of trisomy 3, and recent data obtained by comparative genomic hybridization indicate that the chromosomal regions 3q21-23 and 3q25-29 might be of particular pathogenetic significance. We identified structural chromosomal abnormalities involving the region 3q27 and rearrangements of the BCL6 proto-oncogene in three out of 34 (9%) well-defined cases of extranodal, nodal and splenic MZBCL using cytogenetic analysis, Southern blot, and fluorescence in situ hybridization (FISH). All three cases were characterized by a t(3;14)(q27;q32). Two of them showed additional chromosomal abnormalities including trisomy 3, which was found in one case. The patients displayed extranodal disease and did not demonstrate any striking clinical and histological differences when compared with MZBCL lacking BCL6 rearrangement. The present study for the first time demonstrates the occurrence of t(3;14)/BCL6 gene rearrangement in MZBCL, thus suggesting a role of the BCL6 proto-oncogene in the pathogenesis of MZBCL.

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Frequent association of t(3;14) or variant with other lymphoma‐specific translocations

Cited by 27 publications

References 23 publications

Pathogenetic and Clinical Implications of Non-Immunoglobulin; BCL6 Translocations in B-Cell Non-Hodgkin's Lymphoma

Pathogenetic and Clinical Implications of Non-Immunoglobulin; BCL6 Translocations in B-Cell Non-Hodgkin's Lymphoma

Clinical and biological relevance of single-nucleotide polymorphisms and acquired somatic mutations of the BCL6 first intron in follicular lymphoma

BCL6 gene rearrangements also occur in marginal zone B‐cell lymphoma

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