t(3;7)(q27;p12) IKZF1/BCL6
2005-09-01 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
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
Genes Involved and Proteins
Gene name
BCL6 (B-Cell Lymphoma 6)
Location
3q27.3
Protein description
transcription factor; belongs to the Krüppel family, with a N-term BTB/POZ domain and 6 zinc fingers; transcription repressor
Gene name
IKZF1 (Ikaros family zinc finger 1)
Location
7p12.2
Protein description
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
Description
5 Ikaros - 3 BCL6 fusion transcript; it is supposed that substitution of the promoter of BCL6 may be responsible for BCL6 deregulation
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 8167331 | 1994 | LAZ3 rearrangements in non-Hodgkin's lymphoma: correlation with histology, immunophenotype, karyotype, and clinical outcome in 217 patients. | Bastard C et al |
| 9143685 | 1997 | The role of the Ikaros gene in lymphocyte development and homeostasis. | Georgopoulos K et al |
| 10753856 | 2000 | 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 et al |
| 9648553 | 1998 | A recurrent nonrandom translocation (3;7)(q27;p12) associated with BCL-6 gene rearrangement in B-cell diffuse large cell lymphoma. | Ichinohasama R et al |
| 2328495 | 1990 | Chromosome abnormalities in malignant lymphoma in patients from Kurashiki: histological and immunophenotypic correlations. | Konishi H et al |
| 14596876 | 2003 | Upstream of Ikaros. | Liberg D et al |
| 9618757 | 1998 | Ikaros in hemopoietic lineage determination and homeostasis. | Nichogiannopoulou A et al |
| 12626565 | 2003 | Ikaros isoform x is selectively expressed in myeloid differentiation. | Payne KJ et al |
| 10482987 | 1999 | 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 et al |
Citation
Jean-Loup Huret
t(3;7)(q27;p12) IKZF1/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2005-09-01
Online version: http://atlasgeneticsoncology.org/haematological/2010/t(3;7)(q27;p12)-ikzf1-bcl6
