t(3;8)(q27;q24) BCL6/MYC

2008-03-01   Jean-Loup Huret  

1.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 IDYearTitleCitations
299025762018Lymphomas 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.1

Article Bibliography

Pubmed IDLast YearTitleAuthors
172302272007Mapping of MYC breakpoints in 8q24 rearrangements involving non-immunoglobulin partners in B-cell lymphomas.Bertrand P et al
167721232006A 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
171753792007A 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

Atlas Image
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/tumors-explorer/img/welcome