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 |
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t(3;8)(q27;q24) BCL6/MYC Hybridization with Vysis LSI BCL6 break apart probe (Abbott Molecular, US) showing rearrangement of the gene as a result of t(3;8)(q27;q24) (red signal on der(8) chromosome). Hybridization with Vysis LSI BCL6 break apart and LSI IGH/BCL dual color probes (Abbott Molecular, US) showing rearrangement of BCL6 and IGH as a result of a 3-way t(3;8;14)(q27;q24;q32) - Courtesy Adriana Zamecnikova. | |
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) |
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 |
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