| Written | 1998-03 | Agnes Daudignon |
| Departement d'Hematologie-Immunologie-Cytogenetique, Centre Hospitalier de Valenciennes, France |
| 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 (B-Cell Lymphoma 6) |
| Location | 3q27.3 |
| Dna / Rna | 10 exons |
| Protein | nuclear protein; contains 6 zinc fingers (C2H2 type); transcription factor. |
| Gene Name | RHOH (Ras homolog gene family, member H) |
| Location | 4p14 |
| Protein | expressed in hemopoietic tissues; GTPase of the Ras superfamily; signal transduction. |
| Result of the chromosomal anomaly |
| Description | 5' RHOH - 3' BCL6 and 5' BCL6 - 3' RHOH, leading to two fusion transcripts. |
| 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 |
| Mitelman database | t(3;4)(q27;p13) |
| arrayMap (UZH-SIB Zurich) | [select an item] |
| Mitelman database | RHOH/BCL6 [MCList] RHOH (4p14) BCL6 (3q27.3) |
| REVIEW articles | automatic search in PubMed |
| Last year articles | automatic search in PubMed |
| All articles | automatic search in PubMed |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon Sep 7 15:08:24 CEST 2020 |
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