Atlas of Genetics and Cytogenetics in Oncology and Haematology


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

t(3;7)(q27;p12) IKZF1/BCL6

Written2005-09Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2010

Clinics and Pathology

Disease non Hodgkin lymphoma (NHL)
Phenotype / cell stem origin 2 cases of diffuse large B-cell lymphoma (DLBL) with BCL6/ZNFN1A1 involvement, 1 NHL with BCL6 involvement, 1 follicular, predominantly large cell B-NHL and one T-NHL without gene ascertainement
Epidemiology 5 cases to date, aged 40-70 yrs, sex ration: 3M:1F
Prognosis The 2 patients with BCL6/ZNFN1A1 involvement died at 16 and 17 mths after diagnosis

Cytogenetics

Additional anomalies del(6) in two cases, del(13q) in two cases, +16 in two cases, +12 in one case

Genes involved and Proteins

Gene Name BCL6
Protein transcription factor; belongs to the Krüppel family, with a N-term BTB/POZ domain and 6 zinc fingers; transcription repressor
Gene Name IKZF1
Protein transcription regulator; can repress transcription through the recruitment of histone deacetylase complexes; role in conjunction with Aiolos; hemopoietic-specific zinc finger protein regulator of B and T-cell differentiation

Result of the chromosomal anomaly

Hybrid gene
Description 5' Ikaros - 3' BCL6 fusion transcript; it is supposed that substitution of the promoter of BCL6 may be responsible for BCL6 deregulation
  

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

LAZ3 rearrangements in non-Hodgkin's lymphoma: correlation with histology, immunophenotype, karyotype, and clinical outcome in 217 patients.
Bastard C, Deweindt C, Kerckaert JP, Lenormand B, Rossi A, Pezzella F, Fruchart C, Duval C, Monconduit M, Tilly H
Blood. 1994 ; 83 (9) : 2423-2427.
PMID 8167331
 
The role of the Ikaros gene in lymphocyte development and homeostasis.
Georgopoulos K, Winandy S, Avitahl N
Annual review of immunology. 1997 ; 15 : 155-176.
PMID 9143685
 
The Ikaros gene, a central regulator of lymphoid differentiation, fuses to the BCL6 gene as a result of t(3;7)(q27;p12) translocation in a patient with diffuse large B-cell lymphoma.
Hosokawa Y, Maeda Y, Ichinohasama R, Miura I, Taniwaki M, Seto M
Blood. 2000 ; 95 (8) : 2719-2721.
PMID 10753856
 
A recurrent nonrandom translocation (3;7)(q27;p12) associated with BCL-6 gene rearrangement in B-cell diffuse large cell lymphoma.
Ichinohasama R, Miura I, Funato T, Sato I, Suzuki C, Saito Y, Decoteau JF, Myers JB, Kadin ME, Sawai T, Ooya K
Cancer genetics and cytogenetics. 1998 ; 104 (1) : 19-27.
PMID 9648553
 
Chromosome abnormalities in malignant lymphoma in patients from Kurashiki: histological and immunophenotypic correlations.
Konishi H, Sakurai M, Nakao H, Maseki N, Kaneko Y, Yagiri Y, Notohara K, Frizzera G
Cancer research. 1990 ; 50 (9) : 2698-2703.
PMID 2328495
 
Upstream of Ikaros.
Liberg D, Smale ST, Merkenschlager M
Trends in immunology. 2003 ; 24 (11) : 567-570.
PMID 14596876
 
Ikaros in hemopoietic lineage determination and homeostasis.
Nichogiannopoulou A, Trevisan M, Friedrich C, Georgopoulos K
Seminars in immunology. 1998 ; 10 (2) : 119-125.
PMID 9618757
 
Ikaros isoform x is selectively expressed in myeloid differentiation.
Payne KJ, Huang G, Sahakian E, Zhu JY, Barteneva NS, Barsky LW, Payne MA, Crooks GM
Journal of immunology (Baltimore, Md. : 1950). 2003 ; 170 (6) : 3091-3098.
PMID 12626565
 
Frequent allelic loss of the RB, D13S319 and D13S25 locus in myeloid malignancies with deletion/translocation at 13q14 of chromosome 13, but not in lymphoid malignancies.
Tanaka K, Arif M, Eguchi M, Guo SX, Hayashi Y, Asaoku H, Kyo T, Dohy H, Kamada N
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1999 ; 13 (9) : 1367-1373.
PMID 10482987
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;7)(q27;p12-13)
Atlas Genet Cytogenet Oncol Haematol. 2006;10(2):116-117.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0307q27p12ID2010.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes BCL6 IKZF1

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;7)(q27;p12) IKZF1-BCL6

External links

arrayMap (UZH-SIB Zurich)
Mitelman databaset(3;7)(q27;p12) [Case List]    t(3;7)(q27;p12) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
 
Disease databaset(3;7)(q27;p12) IKZF1/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Thu Jan 12 11:18:17 CET 2017


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.