t(3;4)(q27;p13) RHOH/BCL6

1998-03-01   Agnes Daudignon 

1.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. , superfamily; signal transduction.
Gene name
RHOH (Ras homolog gene family, member H)
Location
4p14
Protein description
expressed in hemopoietic tissues; GTPase of the Ras

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.

Bibliography

Pubmed IDLast YearTitleAuthors
77840611995TTF, 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
103475551999Four 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

External Links