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t(3;16)(q27;p11) IL21R/BCL6

Written2005-09Jean-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 1357
 
  t(3;16)(q27;p11) G- banding - Courtesy Melanie Zenger and Claudia Haferlach.

Clinics and Pathology

Disease Diffuse large cell lymphoma
Epidemiology only one case to date, a 64 yr old male patient
Evolution complete remission was obtained.

Cytogenetics

Cytogenetics Morphological sole anomaly within the subclone

Genes involved and Proteins

Gene Name BCL6
Location 3q27
Dna / Rna spans on 25 kb;. 11 exons
Protein sequence-specific DNA binding transcriptional repressor
Gene Name IL21R
Location 16p11
Dna / Rna spans on 48 kb;. 10 exons
Protein IL21R is, in fact, an heterodimer made of IL21R-alpha, and the common gamma chain (CD132); IL21Ra is the private receptor of IL21; IL21/IL21R, selectively expressed in lymphoid cells, activates the JAK/STAT transcription signaling pathway; IL21/IL21R plays a role in proliferation, differenciation of T cells, B cells, natural killers (NK) cells, and dendritic cells.; promotes and inhibit immune responses

Result of the chromosomal anomaly

Hybrid gene
Description The IL21R promoter region replaces the regulatory sequences of BCL6; the chimeric mRNA consists of the 2 non coding exons 1a/1b of IL21R and the coding exons of BCL6
  
Fusion Protein
Oncogenesis enhanced expression of BCL6
  

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

Translocation (3;16)(q27;p11) in a patient with diffuse large B-cell lymphoma associated with the BCL-6 gene rearrangement.
Ichinohasama R, Miura I, Shishido T, Matsumoto K, Shimizu Y, Miki T, DeCoteau JF, Kadin ME, Ooya K
Cancer genetics and cytogenetics. 1998 ; 103 (2) : 133-139.
PMID 9614912
 
The gene for interleukin-21 receptor is the partner of BCL6 in t(3;16)(q27;p11), which is recurrently observed in diffuse large B-cell lymphoma.
Ueda C, Akasaka T, Kurata M, Maesako Y, Nishikori M, Ichinohasama R, Imada K, Uchiyama T, Ohno H
Oncogene. 2002 ; 21 (3) : 368-376.
PMID 11821949
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;16)(q27;p11)
Atlas Genet Cytogenet Oncol Haematol. 2006;10(2):118-119.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0316q27p11ID1357.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes BCL6 IL21R

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;16)(q27;p11) IL21R/BCL6

External links

arrayMap (UZH-SIB Zurich)IL21R (16p12.1) BCL6 (3q27.3)

IL21R (16p12.1) BCL6 (3q27.3)

IL21R (16p12.1) BCL6 (3q27.3)

Mitelman databaset(3;16)(q27;p11) [Case List]    t(3;16)(q27;p11) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseIL21R/BCL6 [MCList]  IL21R (16p12.1) BCL6 (3q27.3)
Mitelman databaseIL21R/BCL6 [MCList]  IL21R (16p12.1) BCL6 (3q27.3)
TICdbIL21R/BCL6  IL21R (16p12.1) BCL6 (3q27.3)
 
Disease databaset(3;16)(q27;p11) IL21R/BCL6
REVIEW articlesautomatic search in PubMed
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indexed on : Fri Jun 30 11:23:05 CEST 2017


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For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.