Atlas of Genetics and Cytogenetics in Oncology and Haematology


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

t(3;6)(q27;q15) ?/BCL6

Written2012-11Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 1626

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics The t(3;6) was found in two cases of follicular lymphoma (Sanger et al., 1987; Sanchez-Izquierdo et al., 2001).
Prognosis The patient reported by Sanger et al., 1987 was a 83-year-old female patient with a follicular mixed small clived and large cell lymphoma, which evolved to a diffuse mixed small lymphocytic and large cell lymphoma. She experienced persistent disease under treatment, refused further chemotherapy, and died two years after diagnosis.

Cytogenetics

Additional anomalies A t(14;18)(q32;q21) was found in the 2 cases.

Genes involved and Proteins

Note The partner of BCL6 in this translocation is unknown.
Gene Name BCL6
Location 3q27.3
Protein 706 amino acids; composed of a NH2-term BTB/POZ domain (amino acids 1-130 (32-99 according to Swiss-Prot) which mediates homodimerization and protein-protein interactions with other corepressors (including HDAC1 and NCOR2/SMRT to constitute a large repressing complex, another transcription repression domain (191-386), PEST sequences (300-417) with a KKYK motif (375-379), and six zinc finger at the C-term (518-541, 546-568, 574-596, 602-624, 630-652, 658-681), responsible for sequence specific DNA binding. Transcription repressor; recognizes the consensus sequence: TTCCT(A/C)GAA (Albagli-Curiel, 2003). Role in germinal centers of lymphoid follicles. BCL6 prevents ATM and TP53 to induce apoptosis in response to DNA rearrangements such as somatic hypermutation and class switch recombination. Therefore essential for normal B cell development.

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

Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Detection of translocations affecting the BCL6 locus in B cell non-Hodgkin's lymphoma by interphase fluorescence in situ hybridization.
Sanchez-Izquierdo D, Siebert R, Harder L, Marugan I, Gozzetti A, Price HP, Gesk S, Hernandez-Rivas JM, Benet I, Sole F, Sonoki T, Le Beau MM, Schlegelberger B, Dyer MJ, Garcia-Conde J, Martinez-Climent JA.
Leukemia. 2001 Sep;15(9):1475-84.
PMID 11516111
 
Initial and subsequent cytogenetic studies in malignant lymphoma.
Sanger WG, Armitage JO, Bridge J, Weisenburger DD, Fordyce R, Purtilo DT.
Cancer. 1987 Dec 15;60(12):3014-9.
PMID 3315179
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;6)(q27;q15)
Atlas Genet Cytogenet Oncol Haematol. 2013;17(2):134-134.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27q15ID1626.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(3;6)(q27;q15) ?/BCL6

External links

arrayMap (UZH-SIB Zurich)
Mitelman databaset(3;6)(q27;q15) [Case List]    t(3;6)(q27;q15) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
 
Disease databaset(3;6)(q27;q15) ?/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Thu Jan 12 11:18:17 CET 2017


Home   Genes   Leukemias   Solid Tumours   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.