| Written | 2008-03 | Jean-Loup Huret |
| Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France |
| Identity |
| ICD-Topo | C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS |
| Atlas_Id | 2015 |
| Clinics and Pathology |
| Disease | Non Hodgkin lymphoma (NHL) |
| Phenotype / cell stem origin | Found in diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) |
| Epidemiology | 4 cases to date, three cases of DLBCL and one BL. There was 1 male and 3 female patients. Patients were in their fifties (53, 53, and 56 years), apart from an eleven year old patient, but the latter was affected by a cancer-prone disease/chromosome instability syndrome, namely an ataxia telangiectasia (AT). |
| Clinics | The t(3;8) may be considered as a secondary event in two DLBCL cases harbouring a t(14;18) (Bertrand et al, 2007). In the third case of DLBCL, the disease was thought to have evolved from a follicular lymphoma, also present in the patient in various organs (Wang et al, 2007). To be noted that the patient with a BL had AT (Sandlund et al, 2006). |
| Prognosis | Prognostic data were available from only two of the four cases, with survivals noted at: 26+ mths and 114+ mths (in two DLBCL cases). |
| Cytogenetics |
| Cytogenetics Morphological | Major karyotypic abnormalities in 3 of 4 cases, with +7 in two patients, +12 in two cases, t(14;18)(q32;q21) in two cases, and duplication of the der(8)t(3;8) in two cases; Other anomalies known to be recurrent in lymphoid malignancies were also present: del(6q) (one case), and +18 (one case). |
| Genes involved and Proteins |
| Gene Name | BCL6 (B-Cell Lymphoma 6) |
| Location | 3q27.3 |
| Protein | Transcription factor, with a N-term BTB/POZ domain and 6 zinc fingers. The protein can bind to sequence specific DNA and repress its transcription. Normally expressed in germinal center B and T cells |
| Gene Name | MYC v-myc myelocytomatosis viral oncogene homolog (avian) |
| Location | 8q24.21 |
| Protein | Transcription factor, with a DNA binding domain, a helix-loop-helix domain and a leucine zipper. Forms heterodimers with MAX; MYC/MAX complexes bind DNA, activate transcription and promote cell proliferation and transformation. |
| Result of the chromosomal anomaly |
| Description | 5' BCL6 is translocated next to MYC on the der(8) |
| 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 |
| Mapping of MYC breakpoints in 8q24 rearrangements involving non-immunoglobulin partners in B-cell lymphomas. |
| Bertrand P, Bastard C, Maingonnat C, Jardin F, Maisonneuve C, Courel MN, Ruminy P, Picquenot JM, Tilly H. |
| Leukemia. 2007 Mar;21(3):515-23. |
| PMID 17230227 |
| A subtle t(3;8) results in plausible juxtaposition of MYC and BCL6 in a child with Burkitt lymphoma/leukemia and ataxia-telangiectasia. |
| Sandlund JT, Kastan MB, Kennedy W, Behm F, Entrekin E, Pui CH, Kalwinsky DT, Raimondi SC. |
| Cancer Genet Cytogenet. 2006 Jul 1;168(1):69-72. |
| PMID 16772123 |
| A novel t(3;8)(q27;q24.1) simultaneously involving both the BCL6 and MYC genes in a diffuse large B-cell lymphoma. |
| Wang HY, Bossler AD, Schaffer A, Tomczak E, DiPatri D, Frank DM, Nowell PC, Bagg A. |
| Cancer Genet Cytogenet. 2007 Jan 1;172(1):45-53. |
| PMID 17175379 |
| Citation |
| This paper should be referenced as such : |
| Huret, JL |
| t(3;8)(q27;q24) |
| Atlas Genet Cytogenet Oncol Haematol. 2009;13(3):235-236. |
| Free journal version : [ pdf ] [ DOI ] |
| On line version : http://AtlasGeneticsOncology.org/Anomalies/t0308q27q24ID2015.html |
| Other genes implicated (Data extracted from papers in the Atlas) [ 1 ] |
| Genes | BCL6 |
| Translocations implicated (Data extracted from papers in the Atlas) |
| t(3;8)(q27;q24) BCL6/MYC | |
| External links |
| Mitelman database | t(3;8)(q27;q24) [Case List] t(3;8)(q27;q24) [Transloc-MCList] BCL6/MYC [Fusion-MCList] |
| arrayMap (UZH-SIB Zurich) | [select an item] |
| REVIEW articles | automatic search in PubMed |
| Last year articles | automatic search in PubMed |
| All articles | automatic search in PubMed |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon May 14 13:39:31 CEST 2018 |
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