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t(3;9)(q27;p13) GRHPR/BCL6

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

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Atlas_Id 2132

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics The t(3;9)(q27;p13) was found in a case of follicular lymphoma transformed to diffuse aggressive lymphoma, from a study with no individual data (Akasaka et al., 2003), in a 71-year-old female patient with a diagnosis of diffuse large B-cell lymphoma (DLBCL) of stomach, which evolved to a nodular lymphocyte-predominant Hodgkin's lymphoma 3 years later, and to a nodal DLBCL nine years after the initial diagnosis (Wlodarska et al., 2004), in a 47-year-old male patient with Burkitt lymphoma, who died of progressive disease 2 months after diagnosis (Bacher et al., 2011), and in a female patient with a follicular lymphoma (Cheung et al., 2012).


Cytogenetics Morphological The Burkitt lymphoma case showed the characteristic t(8;14)(q24;q32) and a complex karyotype, the follicular lymphoma case showed the t(14;18)(q32;q21). In the DLBCL case, no specific translocation accompanied the t(3;9).

Genes involved and Proteins

Gene NameBCL6 (B-Cell Lymphoma 6)
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 NameGRHPR (glyoxylate and hydroxypyruvate reductase)
Location 9p13.2
Note GRHPR was found involved in the translocation reported in the Akasaka's case, and Wlodarska et al., 2004 also point to its possible involvement.
Protein GRHPR is an enzyme which catalyzes the reduction of hydroxy-pyruvate to D-glycerate, glyoxylate to glycolate and the oxidation of D-glycerate to hydroxypyruvate. Primary hyperoxaluria type 2 is an autosomal recessive disease caused by mutations in GRHPR (Cramer et al., 1999).

Result of the chromosomal anomaly

Hybrid gene
Description Breakpoint in BCL6 first intron.

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.


BCL6 gene translocation in follicular lymphoma: a harbinger of eventual transformation to diffuse aggressive lymphoma.
Akasaka T, Lossos IS, Levy R.
Blood. 2003 Aug 15;102(4):1443-8. Epub 2003 May 8.
PMID 12738680
Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
Several lymphoma-specific genetic events in parallel can be found in mature B-cell neoplasms.
Bacher U, Haferlach T, Alpermann T, Kern W, Schnittger S, Haferlach C.
Genes Chromosomes Cancer. 2011 Jan;50(1):43-50. doi: 10.1002/gcc.20831.
PMID 20960563
SNP analysis of minimally evolved t(14;18)(q32;q21)-positive follicular lymphomas reveals a common copy-neutral loss of heterozygosity pattern.
Cheung KJ, Rogic S, Ben-Neriah S, Boyle M, Connors JM, Gascoyne RD, Horsman DE.
Cytogenet Genome Res. 2012;136(1):38-43. doi: 10.1159/000334265. Epub 2011 Nov 18.
PMID 22104078
The gene encoding hydroxypyruvate reductase (GRHPR) is mutated in patients with primary hyperoxaluria type II.
Cramer SD, Ferree PM, Lin K, Milliner DS, Holmes RP.
Hum Mol Genet. 1999 Oct;8(11):2063-9.
PMID 10484776
Heterogeneity of BCL6 rearrangements in nodular lymphocyte predominant Hodgkin's lymphoma.
Wlodarska I, Stul M, De Wolf-Peeters C, Hagemeijer A.
Haematologica. 2004 Aug;89(8):965-72.
PMID 15339680


This paper should be referenced as such :
Huret, JL
t(3;9)(q27;p13) GRHPR/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2013;17(3):204-205.
Free journal version : [ pdf ]   [ DOI ]
On line version :

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

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;9)(q27;p13) GRHPR/BCL6

External links

GRHPR (9p13.2) BCL6 (3q27.3)

GRHPR (9p13.2) BCL6 (3q27.3)

Mitelman databaset(3;9)(q27;p13) [Case List]    t(3;9)(q27;p13) [Transloc-MCList] GRHPR/BCL6 [Fusion-MCList]
arrayMap (UZH-SIB Zurich)[select an item]
Mitelman databaseGRHPR/BCL6 [MCList]  GRHPR (9p13.2) BCL6 (3q27.3)
Mitelman databaseGRHPR/BCL6 [MCList]  GRHPR (9p13.2) BCL6 (3q27.3)
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