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BCL6 Gene Fusion

MedGen UID:
1669165
•Concept ID:
C4724996
•
Cell or Molecular Dysfunction
Synonyms: B-Cell CLL/Lymphoma 6 Gene Fusion; Bcl-6 Gene Fusion; BCL5 Gene Fusion; LAZ3 Gene Fusion; ZBTB27 Gene Fusion; Zinc Finger Protein 51 Gene Fusion; ZNF51 Gene Fusion

Definition

A molecular genetic abnormality indicating the presence of a fusion gene involving sequences derived from the BCL6 gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBCL6 Gene Fusion

Professional guidelines

PubMed

Decreased CD11c-positive dendritic cells in the tumor microenvironment predict double-hit/triple-hit genotype and survival in diffuse large B-cell lymphoma.
Yuan CT, Chuang SS, Cheng PY, Chang K, Wang H, Tsai JH, Liau JY, Chou WC
J Pathol Clin Res 2022 Sep;8(5):436-447. Epub 2022 Jun 17 doi: 10.1002/cjp2.283. PMID: 35715938Free PMC Article
Identification of "Double Hit" Lymphomas Using Updated WHO Criteria: Insights From Routine MYC Immunohistochemistry in 272 Consecutive Cases of Aggressive B-Cell Lymphomas.
Sakr H, Cook JR
Appl Immunohistochem Mol Morphol 2019 Jul;27(6):410-415. doi: 10.1097/PAI.0000000000000657. PMID: 29629947

Recent clinical studies

Etiology

Emerging molecular subtypes and therapeutic targets in B-cell precursor acute lymphoblastic leukemia.
Li J, Dai Y, Wu L, Zhang M, Ouyang W, Huang J, Chen S
Front Med 2021 Jun;15(3):347-371. Epub 2021 Jan 5 doi: 10.1007/s11684-020-0821-6. PMID: 33400146
Gene of the month: BCOR.
Aldera AP, Govender D
J Clin Pathol 2020 Jun;73(6):314-317. Epub 2020 Mar 11 doi: 10.1136/jclinpath-2020-206513. PMID: 32161069
Clear Cell Sarcoma of the Kidney.
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article
Biology of double-hit B-cell lymphomas.
Lindsley RC, LaCasce AS
Curr Opin Hematol 2012 Jul;19(4):299-304. doi: 10.1097/MOH.0b013e328353bbbd. PMID: 22504522Free PMC Article

Diagnosis

Simplified algorithm for genetic subtyping in diffuse large B-cell lymphoma.
Shen R, Fu D, Dong L, Zhang MC, Shi Q, Shi ZY, Cheng S, Wang L, Xu PP, Zhao WL
Signal Transduct Target Ther 2023 Apr 10;8(1):145. doi: 10.1038/s41392-023-01358-y. PMID: 37032379Free PMC Article
BCL6, a key oncogene, in the placenta, pre-eclampsia and endometriosis.
Louwen F, Kreis NN, Ritter A, Friemel A, Solbach C, Yuan J
Hum Reprod Update 2022 Nov 2;28(6):890-909. doi: 10.1093/humupd/dmac027. PMID: 35640966Free PMC Article
Gene of the month: BCOR.
Aldera AP, Govender D
J Clin Pathol 2020 Jun;73(6):314-317. Epub 2020 Mar 11 doi: 10.1136/jclinpath-2020-206513. PMID: 32161069
Clear Cell Sarcoma of the Kidney.
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
Generation and characterization of a monoclonal antibody against human BCL6 for immunohistochemical diagnosis.
Jia K, Zhang D, Wang Y, Liu Y, Kong X, Yang Q, Chen H, Xie C, Wang S
PLoS One 2019;14(5):e0216470. Epub 2019 May 7 doi: 10.1371/journal.pone.0216470. PMID: 31063496Free PMC Article

Therapy

Simplified algorithm for genetic subtyping in diffuse large B-cell lymphoma.
Shen R, Fu D, Dong L, Zhang MC, Shi Q, Shi ZY, Cheng S, Wang L, Xu PP, Zhao WL
Signal Transduct Target Ther 2023 Apr 10;8(1):145. doi: 10.1038/s41392-023-01358-y. PMID: 37032379Free PMC Article
Inferior survival in high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements is not associated with MYC/IG gene rearrangements.
McPhail ED, Maurer MJ, Macon WR, Feldman AL, Kurtin PJ, Ketterling RP, Vaidya R, Cerhan JR, Ansell SM, Porrata LF, Nowakowski GS, Witzig TE, Habermann TM
Haematologica 2018 Nov;103(11):1899-1907. Epub 2018 Jun 14 doi: 10.3324/haematol.2018.190157. PMID: 29903764Free PMC Article
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article
Integrative epigenomic analysis identifies biomarkers and therapeutic targets in adult B-acute lymphoblastic leukemia.
Geng H, Brennan S, Milne TA, Chen WY, Li Y, Hurtz C, Kweon SM, Zickl L, Shojaee S, Neuberg D, Huang C, Biswas D, Xin Y, Racevskis J, Ketterling RP, Luger SM, Lazarus H, Tallman MS, Rowe JM, Litzow MR, Guzman ML, Allis CD, Roeder RG, Müschen M, Paietta E, Elemento O, Melnick AM
Cancer Discov 2012 Nov;2(11):1004-23. Epub 2012 Oct 29 doi: 10.1158/2159-8290.CD-12-0208. PMID: 23107779Free PMC Article
Biology of double-hit B-cell lymphomas.
Lindsley RC, LaCasce AS
Curr Opin Hematol 2012 Jul;19(4):299-304. doi: 10.1097/MOH.0b013e328353bbbd. PMID: 22504522Free PMC Article

Prognosis

Simplified algorithm for genetic subtyping in diffuse large B-cell lymphoma.
Shen R, Fu D, Dong L, Zhang MC, Shi Q, Shi ZY, Cheng S, Wang L, Xu PP, Zhao WL
Signal Transduct Target Ther 2023 Apr 10;8(1):145. doi: 10.1038/s41392-023-01358-y. PMID: 37032379Free PMC Article
BCL6, a key oncogene, in the placenta, pre-eclampsia and endometriosis.
Louwen F, Kreis NN, Ritter A, Friemel A, Solbach C, Yuan J
Hum Reprod Update 2022 Nov 2;28(6):890-909. doi: 10.1093/humupd/dmac027. PMID: 35640966Free PMC Article
Clear Cell Sarcoma of the Kidney.
Aldera AP, Pillay K
Arch Pathol Lab Med 2020 Jan;144(1):119-123. Epub 2019 Mar 27 doi: 10.5858/arpa.2018-0353-RS. PMID: 30917048
High grade B-cell lymphoma with rearrangements of MYC and BCL2 and/or BCL6: Double hit and triple hit lymphomas and double expressing lymphoma.
Rosenthal A, Younes A
Blood Rev 2017 Mar;31(2):37-42. Epub 2016 Sep 30 doi: 10.1016/j.blre.2016.09.004. PMID: 27717585Free PMC Article
Biology of double-hit B-cell lymphomas.
Lindsley RC, LaCasce AS
Curr Opin Hematol 2012 Jul;19(4):299-304. doi: 10.1097/MOH.0b013e328353bbbd. PMID: 22504522Free PMC Article

Clinical prediction guides

BCL6, a key oncogene, in the placenta, pre-eclampsia and endometriosis.
Louwen F, Kreis NN, Ritter A, Friemel A, Solbach C, Yuan J
Hum Reprod Update 2022 Nov 2;28(6):890-909. doi: 10.1093/humupd/dmac027. PMID: 35640966Free PMC Article
Fusions involving BCOR and CREBBP are rare events in infiltrating glioma.
Pisapia DJ, Ohara K, Bareja R, Wilkes DC, Hissong E, Croyle JA, Kim JH, Saab J, MacDonald TY, Beg S, O'Reilly C, Kudman S, Rubin MA, Elemento O, Sboner A, Greenfield J, Mosquera JM
Acta Neuropathol Commun 2020 Jun 3;8(1):80. doi: 10.1186/s40478-020-00951-4. PMID: 32493417Free PMC Article
Undifferentiated round cell sarcomas with CIC-DUX4 gene fusion: expanding the clinical spectrum.
Brčić I, Brodowicz T, Cerroni L, Kashofer K, Serbanescu GL, Kasseroler MT, Amann G, Scheipl S, Szkandera J, Leithner A, Liegl-Atzwanger B
Pathology 2020 Feb;52(2):236-242. Epub 2019 Dec 20 doi: 10.1016/j.pathol.2019.09.015. PMID: 31870501
Dissecting the mechanism of colorectal tumorigenesis based on RNA-sequencing data.
Liu F, Ji F, Ji Y, Jiang Y, Sun X, Lu Y, Zhang L, Han Y, Liu X
Exp Mol Pathol 2015 Apr;98(2):246-53. Epub 2015 Jan 7 doi: 10.1016/j.yexmp.2015.01.004. PMID: 25576648
Biology of double-hit B-cell lymphomas.
Lindsley RC, LaCasce AS
Curr Opin Hematol 2012 Jul;19(4):299-304. doi: 10.1097/MOH.0b013e328353bbbd. PMID: 22504522Free PMC Article

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