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

Written1998-03Agnes Daudignon
Departement d'Hematologie-Immunologie-Cytogenetique, Centre Hospitalier de Valenciennes, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2055

Clinics and Pathology

Disease NHL
Phenotype / cell stem origin follicular centrocytic-centroblastic lymphoma.
Epidemiology only four cases available to date; all four patients were female; median age: 57 yr.

Cytogenetics

Cytogenetics Morphological in all 4 cases, t(14;18)(q32;q21) was present; t(3;4)(q27;p13) may therefore be a secondary event.
Additional anomalies deletion of 6q was also found in all 4 cases, either as a del(6q) or as a iso(6p); karyotypes were complex.

Genes involved and Proteins

Gene Name BCL6
Location 3q27
Dna / Rna 10 exons
Protein nuclear protein; contains 6 zinc fingers (C2H2 type); transcription factor.
Gene Name RHOH
Location 4p13
Protein expressed in hemopoietic tissues; GTPase of the Ras superfamily; signal transduction.

Result of the chromosomal anomaly

Hybrid gene
Description 5' RHOH - 3' BCL6 and 5' BCL6 - 3' RHOH, leading to two fusion transcripts.
  
Fusion Protein
Description no fusion protein, but promoter exchange between both partner genes.
  

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

TTF, a gene encoding a novel small G protein, fuses to the lymphoma-associated LAZ3 gene by t(3;4) chromosomal translocation.
Dallery E, Galiègue-Zouitina S, Collyn-d'Hooghe M, Quief S, Denis C, Hildebrand MP, Lantoine D, Deweindt C, Tilly H, Bastard C
Oncogene. 1995 ; 10 (11) : 2171-2178.
PMID 7784061
 
Four cases of follicular lymphoma with t(14;18)(q32;q21) and t(3;4)(q27;p13) with LAZ3 (BCL6) rearrangement.
Daudignon A, Bisiau H, Le Baron F, Laï JL, Wetterwald M, Galiègue-Zouitina S, Morel P, Duthilleul P
Cancer genetics and cytogenetics. 1999 ; 111 (2) : 157-160.
PMID 10347555
 

Citation

This paper should be referenced as such :
Daudignon, A
t(3;4)(q27;p13)
Atlas Genet Cytogenet Oncol Haematol. 1998;2(3):94-94.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t34ID2055.html


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

Genes BCL6 RHOH

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;4)(q27;p13) RHOH/BCL6

External links

arrayMap (UZH-SIB Zurich)RHOH (4p14) BCL6 (3q27.3)

Mitelman databaset(3;4)(q27;p13) [Case List]    t(3;4)(q27;p13) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseRHOH/BCL6 [MCList]  RHOH (4p14) BCL6 (3q27.3)
 
Disease databaset(3;4)(q27;p13) RHOH/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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