t(3;8)(q27;q24) BCL6/MYC
2008-03-01 Jean-Loup Huret Affiliation1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
Clinics and Pathology
Disease
Non Hodgkin lymphoma (NHL)
Phenotype stem cell 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).
Genes Involved and Proteins
Gene name
BCL6 (B-Cell Lymphoma 6)
Location
3q27.3
Protein description
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 description
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)
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 32363958 | 2020 | CD56-positive diffuse large B-cell lymphoma/leukemia with BCL6/MYC double-hit and multiple gene mutations: an indicator of poor prognosis? | 23 |
| 29902576 | 2018 | Lymphomas with pseudo-double-hit BCL6-MYC translocations due to t(3;8)(q27;q24) are associated with a germinal center immunophenotype, extranodal involvement, and frequent BCL2 translocations. | 0 |
| 26224092 | 2015 | Clinicopathological features of double-hit B-cell lymphomas with MYC and BCL2, BCL6 or CCND1 rearrangements. | 0 |
| 36167334 | 2022 | Extra copy number of BCL2 is correlated with increased BCL-2 protein expression and poor survival in diffuse large B-cell lymphoma treated with chemoimmunotherapy. | 0 |
| 27124935 | 2016 | Primary Mediastinal Large B-Cell Lymphoma With Translocations Involving BCL6 and MYC (Double-Hit Lymphoma). | 0 |
| 30521492 | 2018 | Two Double-hit Lymphomas Cases: A Molecular Cytogenetic Approach. | 0 |
| 31518494 | 2019 | A large single-institution retrospective analysis of aggressive B-cell lymphomas according to the 2016/2017 WHO classification. | 0 |
| 34438040 | 2021 | SPARC-positive macrophages are the superior prognostic factor in the microenvironment of diffuse large B-cell lymphoma and independent of MYC rearrangement and double-/triple-hit status. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 17230227 | 2007 | Mapping of MYC breakpoints in 8q24 rearrangements involving non-immunoglobulin partners in B-cell lymphomas. | Bertrand P et al |
| 16772123 | 2006 | 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 et al |
| 17175379 | 2007 | 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 et al |
Summary
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.
Citation
Jean-Loup Huret
t(3;8)(q27;q24) BCL6/MYC
Atlas Genet Cytogenet Oncol Haematol. 2008-03-01
Online version: http://atlasgeneticsoncology.org/haematological/2015/t(3;8)(q27;q24)-bcl6-myc
