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t(3;3)(q27;q29) TFRC/BCL6

Written2009-05Jean-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 2087

Clinics and Pathology

Disease Non Hodgkin lymphoma (NHL).
Epidemiology Four cases to date: a follicular mixed cell lymphoma in a 78-year-old female patient, a grade 3B follicular lymphoma in a female patient, a gastric lymphoma, and a diffuse large B-cell lymphoma (DLCL) (Yunis et al., 1984; Bosga-Bouwer et al., 2003; Chen et al., 2006; Yoshida et al., 1999).
Prognosis No data.

Cytogenetics

Additional anomalies There was a complex karyotype, including a t(14;18)(q32;q21), +15, and other anomalies in one case (Yunis et al., 1984), and also a complex karyotype in an other case (Bosga-Bouwer et al., 2003); data is missing in the two other cases.

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).
Gene NameTFRC (transferrin receptor (p90, CD71))
Location 3q29
Protein 760 amino acids; composed of an NH2-term cytoplasmic domain (amino acids 1-67), a transmembrane domain (aa 68-88), and a C term extracellular domain (aa 89-706) according to Swiss-Prot. Cell surface membrane glycoprotein; involved in iron homeostasis by regulating cellular iron uptake (Dorak, 2008).

Result of the chromosomal anomaly

Hybrid gene
Description 5' TFRC - 3' BCL6, with a breakpoint in BCL6 between exon 1 and 2 (Yoshida et al., 1999); the breakpoint on TFRC was found 288 bp 3' downstream of the breakpoint in the other case studied with molecular techniques (Chen et al., 2006).
  

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
 
Follicular lymphoma grade 3B includes 3 cytogenetically defined subgroups with primary t(14;18), 3q27, or other translocations: t(14;18) and 3q27 are mutually exclusive.
Bosga-Bouwer AG, van Imhoff GW, Boonstra R, van der Veen A, Haralambieva E, van den Berg A, de Jong B, Krause V, Palmer MC, Coupland R, Kluin PM, van den Berg E, Poppema S.
Blood. 2003 Feb 1;101(3):1149-54.
PMID 12529293
 
High BCL6 expression predicts better prognosis, independent of BCL6 translocation status, translocation partner, or BCL6-deregulating mutations, in gastric lymphoma.
Chen YW, Hu XT, Liang AC, Au WY, So CC, Wong ML, Shen L, Tao Q, Chu KM, Kwong YL, Liang RH, Srivastava G.
Blood. 2006 Oct 1;108(7):2373-83. Epub 2006 Jun 13.
PMID 16772602
 
TFRC (transferrin receptor (p90, CD71)).
Dorak MT.
Atlas Genet Cytogenet Oncol Haematol. April 2008. URL: http://AtlasGeneticsOncology.org/Genes/TFRCID259ch3q29.html.
 
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
 
Recurrent chromosomal defects are found in most patients with non-Hodgkin's-lymphoma.
Yunis JJ, Oken MM, Theologides A, Howe RB, Kaplan ME.
Cancer Genet Cytogenet. 1984 Sep;13(1):17-28.
PMID 6467179
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;3)(q27;q29)
Atlas Genet Cytogenet Oncol Haematol. 2010;14(4):431-432.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0303q27q29ID2087.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;3)(q27;q29) TFRC/BCL6

External links

arrayMap (UZH-SIB Zurich)TFRC (3q29) BCL6 (3q27.3)

TFRC (3q29) BCL6 (3q27.3)

Mitelman databaset(3;3)(q27;q29) [Case List]    t(3;3)(q27;q29) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseTFRC/BCL6 [MCList]  TFRC (3q29) BCL6 (3q27.3)
TICdbTFRC/BCL6  TFRC (3q29) BCL6 (3q27.3)
 
Disease databaset(3;3)(q27;q29) TFRC/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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