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t(3;6)(q27;p22) HIST1H4I/BCL6

Written2012-12Jean-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 2009

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics Apparently 6 cases have been described: a case of follicular mixed small cleaved and large cell lymphoma, a case of follicular large cell lymphoma, and 3 cases of diffuse large B-cell lymphoma (Akasaka et al., 1997; Akasaka et al., 2000; Kurata et al., 2002; Ohno, 2006); and a case of primary central nervous system lymphoma (PCNSL, a diffuse large B cell lymphoma confined to the brain) (Schwindt et al., 2006).

Cytogenetics

Cytogenetics Morphological Only two of the five cases reported by the Kyoto group showed a t(3;6)(q27;p21-22).

Genes involved and Proteins

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.
Gene Name HIST1H4I
Location 6p22.1
Protein Component of the nucleosome. Histones play a major role in DNA repair, replication and transcription.

Result of the chromosomal anomaly

Hybrid gene
Description Breakpoints in HIST1H4 are located within the single exon to 3' of the terminal palindrome; the breakpoint in BCL6 was located within the major translocation cluster.
  

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

Molecular anatomy of BCL6 translocations revealed by long-distance polymerase chain reaction-based assays.
Akasaka H, Akasaka T, Kurata M, Ueda C, Shimizu A, Uchiyama T, Ohno H.
Cancer Res. 2000 May 1;60(9):2335-41.
PMID 10811103
 
A recurring translocation, t(3;6)(q27;p21), in non-Hodgkin's lymphoma results in replacement of the 5' regulatory region of BCL6 with a novel H4 histone gene.
Akasaka T, Miura I, Takahashi N, Akasaka H, Yonetani N, Ohno H, Fukuhara S, Okuma M.
Cancer Res. 1997 Jan 1;57(1):7-12.
PMID 8988030
 
Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Characterization of t(3;6)(q27;p21) breakpoints in B-cell non-Hodgkin's lymphoma and construction of the histone H4/BCL6 fusion gene, leading to altered expression of Bcl-6.
Kurata M, Maesako Y, Ueda C, Nishikori M, Akasaka T, Uchiyama T, Ohno H.
Cancer Res. 2002 Nov 1;62(21):6224-30.
PMID 12414651
 
Pathogenetic and clinical implications of non-immunoglobulin ; BCL6 translocations in B-cell non-Hodgkin's lymphoma.
Ohno H.
J Clin Exp Hematop. 2006 Nov;46(2):43-53. (REVIEW)
PMID 17142954
 
Chromosomal translocations fusing the BCL6 gene to different partner loci are recurrent in primary central nervous system lymphoma and may be associated with aberrant somatic hypermutation or defective class switch recombination.
Schwindt H, Akasaka T, Zuhlke-Jenisch R, Hans V, Schaller C, Klapper W, Dyer MJ, Siebert R, Deckert M.
J Neuropathol Exp Neurol. 2006 Aug;65(8):776-82.
PMID 16896311
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;6)(q27;p22) HIST1H4I/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2013;17(4):277-278.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27p22ID2009.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;6)(q27;p22) HIST1H4I/BCL6

External links

arrayMap (UZH-SIB Zurich)HIST1H4I (6p22.1) BCL6 (3q27.3)

HIST1H4I (6p22.1) BCL6 (3q27.3)

HIST1H4I (6p22.1) BCL6 (3q27.3)

Mitelman databaset(3;6)(q27;p22) [Case List]    t(3;6)(q27;p22) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseHIST1H4I/BCL6 [MCList]  HIST1H4I (6p22.1) BCL6 (3q27.3)
Mitelman databaseHIST1H4I/BCL6 [MCList]  HIST1H4I (6p22.1) BCL6 (3q27.3)
TICdbHIST1H4I/BCL6  HIST1H4I (6p22.1) BCL6 (3q27.3)
 
Disease databaset(3;6)(q27;p22) HIST1H4I/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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