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

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
Location	3q27
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
Location	8q24
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

Hybrid gene

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

arrayMap (UZH-SIB Zurich)

Mitelman database	t(3;8)(q27;q24) [Case List]    t(3;8)(q27;q24) [Association List] Mitelman database (CGAP - NCBI)
arrayMap	[select an item]
Disease database	t(3;8)(q27;q24) BCL6/MYC

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indexed on : Fri Jun 30 11:23:01 CEST 2017

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