| 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 (or IL21R-alpha) |
| 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 |
| 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 |
| Oncogenesis | enhanced expression of BCL6 |
| External links |
| Other database | t(3;16)(q27;p11) | Mitelman database (CGAP - NCBI) | |
| Other database | t(3;16)(q27;p11) | 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 |
| 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 |
| Contributor(s) |
| Written | 09-2005 | Jean Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . t(3;16)(q27;p11). Atlas Genet Cytogenet Oncol Haematol. Septem
ber 2005 . URL : http://AtlasGeneticsOncology.org/Genes/t0316q27p11ID1357.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Wed Sep 24 21:06:35 2008 |
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