t(3;4)(q27;p13) RHOH/BCL6
1998-03-01 Agnes Daudignon Affiliation1.Departement dHematologie-Immunologie-Cytogenetique, Centre Hospitalier de Valenciennes, France
Clinics and Pathology
Disease
NHL
Phenotype stem cell origin
follicular centrocytic-centroblastic lymphoma.
Epidemiology
only four cases available to date; all four patients werefemale; median age: 57 yr.
Cytogenetics
Cytogenetics morphological
in all 4 cases, t(14;18)(q32;q21) was present; t(3;4)(q27;p13) maytherefore be a secondary event.
Additional anomalies
deletion of 6q was also found in all 4 cases, eitheras a del(6q) or as a iso(6p); karyotypes were complex.
Genes Involved and Proteins
Gene name
BCL6 (B-Cell Lymphoma 6)
Location
3q27.3
Dna rna description
10 exons
Protein description
nuclear protein; contains 6 zinc fingers (C2H2 type);transcription factor.
Gene name
RHOH (Ras homolog gene family, member H)
Location
4p14
Protein description
expressed in hemopoietic tissues; GTPase of the Ras
Protein description
superfamily; signal transduction.
Result of the Chromosomal Anomaly
Description
5 RHOH - 3 BCL6 and 5 BCL6 - 3 RHOH, leading to two fusiontranscripts.no fusion protein, but promoter exchange between both partner genes.
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 7784061 | 1995 | TTF, a gene encoding a novel small G protein, fuses to the lymphoma-associated LAZ3 gene by t(3;4) chromosomal translocation. | Dallery E et al |
| 10347555 | 1999 | Four cases of follicular lymphoma with t(14;18)(q32;q21) and t(3;4)(q27;p13) with LAZ3 (BCL6) rearrangement. | Daudignon A et al |
Summary
Fusion gene
RHOH/BCL6 RHOH (4p14) BCL6 (3q27.3) M t(3;4)(q27;p14)
Citation
Agnes Daudignon
t(3;4)(q27;p13) RHOH/BCL6
Atlas Genet Cytogenet Oncol Haematol. 1998-03-01
Online version: http://atlasgeneticsoncology.org/haematological/2055/t(3;4)(q27;p13)-rhoh-bcl6
