t(3;16)(q27;p13) CIITA/BCL6

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Written2006-03Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France

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1. Identity

Atlas_Id 2089

2. Clinics and Pathology

Disease B cell non Hodgkin lymphoma (NHL)
Epidemiology Only 3 cases so far
Clinics The 3 patients had diffuse large B cell lymphoma (DLBCL); 2 of 2 patients were male patients, one presented with a history of follicular small cleaved cell lymphoma 15 yrs ago, when aged 47 yr. This patient died 6 mths later of progressive disease.

3. Cytogenetics

Additional anomalies 2 out of 2 cases had a t(14;18)(q32;q21) and both patients presented with MAKA (major karyotypic anomalies)

4. Genes involved and Proteins

Gene NameBCL6 (B-Cell Lymphoma 6)
Location 3q27.3
Dna / Rna spans on 25 kb;. 11 exons
Protein Sequence-specific DNA binding transcriptional repressor
Gene NameCIITA (MHC class II transactivator)
Location 16p13.13
Protein Non-DNA-binding transcriptional coactivator; transactivator of the major histocompatibility complex (MHC) class II genes

5. Result of the chromosomal anomaly

Hybrid gene
Description 5'-MHC2TA/BCL6-3' fusion transcripts but also the 5'-BCL6/MHC2TA-3' fusion transcripts,

6. 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.

7. Bibliography

Concurrent chromosomal alterations at 3q27, 8q24 and 18q21 in B-cell lymphomas.
Au WY, Gascoyne RD, Viswanatha DS, Skinnider BF, Connors JM, Klasa RJ, Horsman DE
British journal of haematology. 1999 ; 105 (2) : 437-440.
PMID 10233417
Detection of reciprocal fusion 5'-BCL6/partner-3' transcripts in lymphomas exhibiting reciprocal BCL6 translocations.
Kaneita Y, Yoshida S, Ishiguro N, Sawada U, Horie T, Mori S, Moriyama M
British journal of haematology. 2001 ; 113 (3) : 803-806.
PMID 11380473
Detection of translocations affecting the BCL6 locus in B cell non-Hodgkin's lymphoma by interphase fluorescence in situ hybridization.
Sanchez-Izquierdo D, Siebert R, Harder L, Marugan I, Gozzetti A, Price HP, Gesk S, Hernandez-Rivas JM, Benet I, Solé F, Sonoki T, Le Beau MM, Schlegelberger B, Dyer MJ, Garcia-Conde J, Martinez-Climent JA
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2001 ; 15 (9) : 1475-1484.
PMID 11516111
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 ; 18 (56) : 7994-7999.
PMID 10637510

8. Citation

This paper should be referenced as such :
Huret, JL
Atlas Genet Cytogenet Oncol Haematol. 2006;10(3):197-197.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://atlasgeneticsoncology.usal.es/classic/Anomalies/t0316q27p13ID2089.html

9. Translocations implicated (Data extracted from papers in the Atlas)

 t(3;16)(q27;p13) CIITA/BCL6

10. External links

CIITA (16p13.13) BCL6 (3q27.3)

Mitelman databaset(3;16)(q27;p13) [Case List]    t(3;16)(q27;p13) [Transloc-MCList] CIITA/BCL6 [Fusion-MCList]
arrayMap (UZH-SIB Zurich)[select an item]
Mitelman databaseCIITA/BCL6 [MCList]  CIITA (16p13.13) BCL6 (3q27.3)
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed

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indexed on : Wed Nov 28 16:18:18 CET 2018

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