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

2006-03-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

T-cell lymphoma of the small intestine of low grade malignancy

Phenotype stem cell origin

CD3, CD4, TCR A-B phenotype.

Epidemiology

Only 1 case to date, a 28-year-old male patient.

Genes Involved and Proteins

Gene name
IL2 (interleukin 2)
Location
4q27
Dna rna description
6 exons
Protein description
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 name
TNFRSF17 (tumor necrosis factor receptor superfamily, member 17)
Location
16p13.13
Dna rna description
3 exons
Protein description
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

Description

5 IL2 - 3 TNFRSF17; breakpoint in intron 3 of IL2.

Bibliography

Pubmed IDLast YearTitleAuthors
83133321994Extensive small intestinal T-cell lymphoma of low-grade malignancy associated with a new chromosomal translocation.Carbonnel F et al
13965831992A 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 et al

Summary

Fusion gene

IL2/TNFRSF17 IL2 (4q27) TNFRSF17 (16p13.13) M|IL2/TNFRSF17 IL2 (4q27) TNFRSF17 (16p13.13) M t(4;16)(q27;p13)

Citation

Jean-Loup Huret

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

Atlas Genet Cytogenet Oncol Haematol. 2006-03-01

Online version: http://atlasgeneticsoncology.org/haematological/1358/t(4;16)(q26;p13)

External Links