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t(4;16)(q26;p13) IL2/TNFRSF17

Written2006-03Jean-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 1358

Clinics and Pathology

Disease T-cell lymphoma of the small intestine of low grade malignancy
Phenotype / cell stem origin CD3, CD4, TCR A-B phenotype.
Epidemiology Only 1 case to date, a 28-year-old male patient.

Cytogenetics

Cytogenetics Morphological Sole anomaly.

Genes involved and Proteins

Gene NameIL2 (interleukin 2)
Location 4q27
Dna / Rna 6 exons
Protein Role in mediation of the immune system; member of the IL2 family of cytokines; binds to and signals through a receptor complex consisting of IL2Ralpha, IL2Rbeta, and gamma chain; T-cell growth factor.
Gene NameTNFRSF17 (tumor necrosis factor receptor superfamily, member 17)
Location 16p13.13
Dna / Rna 3 exons
Protein Role in mediation of the immune system; member of the TNF-receptor superfamily; binds to TNFSF13B/BAFF/BLys and TNFSF13/APRIL; promotes B-cell survival.

Result of the chromosomal anomaly

Hybrid gene
Description 5' IL2 - 3' TNFRSF17; breakpoint in intron 3 of IL2.
  

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

Extensive small intestinal T-cell lymphoma of low-grade malignancy associated with a new chromosomal translocation.
Carbonnel F, Lavergne A, Messing B, Tsapis A, Berger R, Galian A, Nemeth J, Brouet JC, Rambaud JC
Cancer. 1994 ; 73 (4) : 1286-1291.
PMID 8313332
 
A new gene, BCM, on chromosome 16 is fused to the interleukin 2 gene by a t(4;16)(q26;p13) translocation in a malignant T cell lymphoma.
Laâbi Y, Gras MP, Carbonnel F, Brouet JC, Berger R, Larsen CJ, Tsapis A
The EMBO journal. 1992 ; 11 (11) : 3897-3904.
PMID 1396583
 

Citation

This paper should be referenced as such :
Huret, JL
t(4;16)(q26;p13)
Atlas Genet Cytogenet Oncol Haematol. 2006;10(3):198-198.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0416q26p13ID1358.html


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

Genes TNFRSF17

Translocations implicated (Data extracted from papers in the Atlas)

 t(4;16)(q26;p13) IL2/TNFRSF17

External links

arrayMap (UZH-SIB Zurich)IL2 (4q27) TNFRSF17 (16p13.13)

IL2 (4q27) TNFRSF17 (16p13.13)

Mitelman databaset(4;16)(q26;p13) [Case List]    t(4;16)(q26;p13) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseIL2/TNFRSF17 [MCList]  IL2 (4q27) TNFRSF17 (16p13.13)
Mitelman databaseIL2/TNFRSF17 [MCList]  IL2 (4q27) TNFRSF17 (16p13.13)
 
Disease databaset(4;16)(q26;p13) IL2/TNFRSF17
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Tue Nov 21 15:09:26 CET 2017


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