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t(3;14)(q27;q32) IGH/BCL6

t(2;3)(p12;q27) IGK/BCL6

t(3;22)(q27;q11) IGL/BCL6

Written1998-04Christian Bastard
Department of Hematology, Centre Henri Becquerel, Rouen, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2004
Note the three translocations are variant of each other, share identical features, and represent translocations of the same proto-oncogene into various immunoglobulin loci.
 
  t(2;3)(p12;q27) G- banding (above) - Courtesy Jean-Luc Lai and Alain Vanderhaegen; t(3;14)(q27;q32) G-banding (middle) and t(3;22)(q27;q11) G-banding (below) - Courtesy Diane H. Norback, Eric B. Johnson, and Sara Morrison-Delap, UW Cytogenetic Services

Clinics and Pathology

Disease NHL; mainly diffuse large cell lymphoma (DLCL); high grade lymphoma; rare cases with low grade/follicular lymphoma; in those cases, 3q rearrangements can be isolated or associated with a t(14;18) translocation
Phenotype / cell stem origin B-cell
Epidemiology 3q27 rearrangements present in 25 to 30 % of DLCL; about 10 % of follicular lymphoma
Clinics mainly adult aggressive lymphoma
Treatment intensive chemotherapy
Prognosis controversial

Cytogenetics

Cytogenetics Morphological t(2;3) and t(3;22) are easily identified; t(3 ;14) is telomeric and difficult to see, especially when using G banding
Cytogenetics Molecular some cosmid and YAC probes have been developed
Additional anomalies very frequent, as usual in NHL, involving chromosome 1, del(6q), 10q, 11q, 12q, 13q, 17p arms
Variants large number of recurring defects involving the 3q27 region: t(3;4)(q27;p13), t(3;6)(q27;p22), t(3;7)(q27;p13), t(3;8)(q27;q24), t(3;11)(q27;q23), t(3;13)(q27;q14), t(3;15)(q27;q22), t(3;17)(q27;q11).

Genes involved and Proteins

Note BCL 6 (see below) with various partners: immunoglobulin genes IgH (mainly Sμ region) in 14q32, IgK in 2p12, and IgL in 22q11; (for variant translocations, partners are: RHOH in the t(3;4), histone H1F1 in the t(3;6), OBF1 in the t(3;11), LCP1 in the t(3;13); in these cases, the first non-coding exon of the partner gene fuses with the second exon of BCL6, resulting in the deregulated expression of a chimerical transcript and the production of a normal BCL6 protein)
Gene NameBCL6 (B-Cell Lymphoma 6)
Location 3q27.3
Dna / Rna 10 exons; alternative splicing of exons 1 (1a and 1b), without modification of the open reading frame
Protein transcription factor; belongs to the Krüppel family, with a N-term BTB/POZ domain and 6 zinc fingers; transcription repressor.

Result of the chromosomal anomaly

Fusion Protein
Description no fusion protein, but promoter exchange; the immunoglobulin gene promoter controls the expression of the BCL6 gene.
Oncogenesis deregulated protein expression; alternative deregulation mechanisms: deletion of regulating sequences, mutations.
  

Bibliography

Identification of the gene associated with the recurring chromosomal translocations t(3;14)(q27;q32) and t(3;22)(q27;q11) in B-cell lymphomas.
Baron BW, Nucifora G, McCabe N, Espinosa R 3rd, Le Beau MM, McKeithan TW
Proceedings of the National Academy of Sciences of the United States of America. 1993 ; 90 (11) : 5262-5266.
PMID 8506375
 
LAZ3 rearrangements in non-Hodgkin's lymphoma: correlation with histology, immunophenotype, karyotype, and clinical outcome in 217 patients.
Bastard C, Deweindt C, Kerckaert JP, Lenormand B, Rossi A, Pezzella F, Fruchart C, Duval C, Monconduit M, Tilly H
Blood. 1994 ; 83 (9) : 2423-2427.
PMID 8167331
 
Translocations involving band 3q27 and Ig gene regions in non-Hodgkin's lymphoma.
Bastard C, Tilly H, Lenormand B, Bigorgne C, Boulet D, Kunlin A, Monconduit M, Piguet H
Blood. 1992 ; 79 (10) : 2527-2531.
PMID 1586704
 
Cloning of a breakpoint cluster region at band 3q27 involved in human non-Hodgkin's lymphoma.
Deweindt C, Kerckaert JP, Tilly H, Quief S, Nguyen VC, Bastard C
Genes, chromosomes & cancer. 1993 ; 8 (3) : 149-154.
PMID 7509622
 
LAZ3, a novel zinc-finger encoding gene, is disrupted by recurring chromosome 3q27 translocations in human lymphomas.
Kerckaert JP, Deweindt C, Tilly H, Quief S, Lecocq G, Bastard C
Nature genetics. 1993 ; 5 (1) : 66-70.
PMID 8220427
 
Gene involved in the 3q27 translocation associated with B-cell lymphoma, BCL5, encodes a Krüppel-like zinc-finger protein.
Miki T, Kawamata N, Hirosawa S, Aoki N
Blood. 1994 ; 83 (1) : 26-32.
PMID 8274740
 
Rearrangement of the bcl-6 gene as a prognostic marker in diffuse large-cell lymphoma.
Offit K, Lo Coco F, Louie DC, Parsa NZ, Leung D, Portlock C, Ye BH, Lista F, Filippa DA, Rosenbaum A
The New England journal of medicine. 1994 ; 331 (2) : 74-80.
PMID 8208268
 
Alterations of a zinc finger-encoding gene, BCL-6, in diffuse large-cell lymphoma.
Ye BH, Lista F, Lo Coco F, Knowles DM, Offit K, Chaganti RS, Dalla-Favera R
Science (New York, N.Y.). 1993 ; 262 (5134) : 747-750.
PMID 8235596
 
Cloning of bcl-6, the locus involved in chromosome translocations affecting band 3q27 in B-cell lymphoma.
Ye BH, Rao PH, Chaganti RS, Dalla-Favera R
Cancer research. 1993 ; 53 (12) : 2732-2735.
PMID 8504412
 

Citation

This paper should be referenced as such :
Bastard, C
t(3;14)(q27;q32) - t(2;3)(p12;q27) - t(3;22)(q27;q11)
Atlas Genet Cytogenet Oncol Haematol. 1998;2(4):122-124.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t314ID2004.html


Other genes implicated (Data extracted from papers in the Atlas) [ 7 ]

Genes BCL6 BCL6 BCL6 BCL6 IGH IGK IGL

Translocations implicated (Data extracted from papers in the Atlas)

 t(2;3)(p12;q27)
 t(3;14)(q27;q32)
 t(3;22)(q27;q11) IGL/BCL6

External links

arrayMap (UZH-SIB Zurich)IGL (22q11.22) BCL6 (3q27.3)

Mitelman databaset(2;3)(p12;q27) [Case List]    t(2;3)(p12;q27) [Association List] Mitelman database (CGAP - NCBI)
Mitelman databaset(3;14)(q27;q32) [Case List]    t(3;14)(q27;q32) [Association List] Mitelman database (CGAP - NCBI)
Mitelman databaset(3;22)(q27;q11) [Case List]    t(3;22)(q27;q11) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseIGL/BCL6 [MCList]  IGL (22q11.22) BCL6 (3q27.3)
 
Disease databaset(3;14)(q27;q32) IGH/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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