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t(3;6)(q27;p21)

Written2009-08Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2155
Note In only two cases were the hybrid genes uncovered: a PIM1/BCL6 was found in one case (Yoshida et al., 1999), and a SFRS3/BCL6 in another case (Chen et al., 2001). These cases will also be described separately.

Clinics and Pathology

Disease Non Hodgkin lymphoma (NHL).
Epidemiology Seven cases available: 3 cases of follicular lymphoma (FL), 3 cases of diffuse large B-cell lymphoma (DLBCL), and one NHL not otherwise specified. Very few data available: 4 male / 2 female patients, aged 49-79 years (Ohno et al., 1994; Miura et al., 1996; Yoshida et al., 1999; Chen et al., 2001; Varga et al., 2001; Keller et al., 2006).

Cytogenetics

Cytogenetics Morphological The t(3;6) was the sole anomaly in one case (a FL case), accompanied with +X in a subclone and +21 in another in the other FL case, found within a complex karyotype in the 4 remainind cases with cytogenetic data. Other additional anomalies of note were: del(7q), t(14;18)(q32;q21) (2 cases each) and t(1;14)(q21;q32), del(1q), del(5q) (1 case each).

Genes involved and Proteins

Gene NameBCL6 (B-Cell Lymphoma 6)
Location 3q27.3
Protein 706 amino acids; composed of a NH2-term BTB/POZ domain (amino acids 1-130 (32-99 according to Swiss-Prot)) which mediates homodimerization and protein-protein interactions with other corepressors (including HDAC1 and NCOR2/SMRT) to constitute a large repressing complex, another transcription repression domain (191-386), PEST sequences (300-417) with a KKYK motif (375-379), and six zinc finger at the C-term (518-541, 546-568, 574-596, 602-624, 630-652, 658-681), responsible for sequence specific DNA binding. Transcription repressor; recognizes the consensus sequence: TTCCT(A/C)GAA (Albagli-Curiel, 2003).

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Splicing factor SRP20 is a novel partner of BCL6 in a t(3;6)(q27;p21) translocation in transformed follicular lymphoma.
Chen W, Itoyama T, Chaganti RS.
Genes Chromosomes Cancer. 2001 Nov;32(3):281-4.
PMID 11579468
 
Intrachromosomal rearrangement of chromosome 3q27: an under recognized mechanism of BCL6 translocation in B-cell non-Hodgkin lymphoma.
Keller CE, Nandula S, Vakiani E, Alobeid B, Murty VV, Bhagat G.
Hum Pathol. 2006 Aug;37(8):1093-9. Epub 2006 Jun 21.
PMID 16867873
 
A new non-random chromosomal translocation t(3;6)(q27;p21.3) associated with BCL6 rearrangement in two patients with non-Hodgkin's lymphoma.
Miura I, Ohshima A, Takahashi N, Hashimoto K, Nimura T, Utsumi S, Saito M, Miki T, Hirosawa S, Miura AB.
Int J Hematol. 1996 Oct;64(3-4):249-56.
PMID 8923787
 
Heterogeneity in B-cell neoplasms associated with rearrangement of the LAZ3 gene on chromosome band 3q27.
Ohno H, Kerckaert JP, Bastard C, Fukuhara S.
Jpn J Cancer Res. 1994 Jun;85(6):592-600.
PMID 8063612
 
Clustering of 1p36 breakpoints distal to 1p36.2 in hematological malignancies.
Varga AE, Dobrovic A, Webb GC, Hutchinson R.
Cancer Genet Cytogenet. 2001 Feb;125(1):78-9.
PMID 11303527
 
Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples.
Yoshida S, Kaneita Y, Aoki Y, Seto M, Mori S, Moriyama M.
Oncogene. 1999 Dec 23;18(56):7994-9.
PMID 10637510
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;6)(q27;p21)
Atlas Genet Cytogenet Oncol Haematol. 2010;14(7):692-693.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27p21ID2155.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(3;6)(q27;p21)

External links

arrayMap (UZH-SIB Zurich)
Mitelman databaset(3;6)(q27;p21) [Case List]    t(3;6)(q27;p21) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
 
Disease databaset(3;6)(q27;p21)
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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