| 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 Name | IL2 |
| Location | 4q26 |
| 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 Name | TNFRSF17 |
| Location | 16p13.1 |
| 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 |
| Description | 5' IL2 - 3' TNFRSF17; breakpoint in intron 3 of IL2. |
| External links |
| Other database | t(4;16)(q26;p13) | Mitelman database (CGAP - NCBI) | |
| Other database | t(4;16)(q26;p13) | CancerChromosomes (NCBI) |
| 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 |
| 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. |
| Laˆ¢bi Y, Gras MP, Carbonnel F, Brouet JC, Berger R, Larsen CJ, Tsapis A |
| The EMBO journal. 1992 ; 11 (11) : 3897-3904. |
| PMID 1396583 |
| 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 |
| Contributor(s) |
| Written | 03-2006 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . t(4;16)(q26;p13). Atlas Genet Cytogenet Oncol Haematol. March 2006 . URL : http://AtlasGeneticsOncology.org/Anomalies/t0416q26p13ID1358.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Sat Feb 6 15:45:47 CET 2010 |
For comments and suggestions or contributions, please contact us