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BCL6 Gene Rearrangement

MedGen UID:
1379454
•Concept ID:
C0805251
•
Cell or Molecular Dysfunction
Synonyms: B-Cell CLL/Lymphoma 6 Gene Rearrangement; Bcl-6 Gene Rearrangement; BCL5 Gene Rearrangement; LAZ3 Gene Rearrangement; ZBTB27 Gene Rearrangement; Zinc Finger Protein 51 Gene Rearrangement; ZNF51 Gene Rearrangement

Definition

A molecular abnormality indicating rearrangement of the BCL6 gene. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Molecularly Stratified Treatment Options in Primary Refractory DLBCL/HGBL with MYC and BCL2 or BCL6 Rearrangements (HGBL, NOS with MYC/BCL6).
Witte HM, Riedl J, Künstner A, Fähnrich A, Ketzer J, Fliedner SMJ, Reimer N, Bernard V, von Bubnoff N, Merz H, Busch H, Feller A, Gebauer N
Target Oncol 2023 Sep;18(5):749-765. Epub 2023 Jul 24 doi: 10.1007/s11523-023-00983-5. PMID: 37488307Free PMC Article
Double hit and double expressors in lymphoma: Definition and treatment.
Riedell PA, Smith SM
Cancer 2018 Dec 15;124(24):4622-4632. Epub 2018 Sep 25 doi: 10.1002/cncr.31646. PMID: 30252929
Management of Patients with MYC-Altered Lymphomas.
Landsburg DJ
Curr Hematol Malig Rep 2016 Jun;11(3):208-17. doi: 10.1007/s11899-016-0320-7. PMID: 26983958

Recent clinical studies

Etiology

Characterisation of immunogenotypes of diffuse large B-cell lymphoma.
Phang KC, Hussin NH, Abdul Rahman F, Tizen NMS, Mansoor A, Masir N
Malays J Pathol 2019 Aug;41(2):101-124. PMID: 31427546
Clinical Significance of BCL2, C-MYC, and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC/BCL2 Proteins and Co-expression of MYC/BCL2/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients.
Ting CY, Chang KM, Kuan JW, Sathar J, Chew LP, Wong OJ, Yusuf Y, Wong L, Samsudin AT, Pana MNBM, Lee SK, Gopal NSR, Puri R, Ong TC, Bahari SK, Goh AS, Teoh CS
Int J Med Sci 2019;16(4):556-566. Epub 2019 Apr 20 doi: 10.7150/ijms.27610. PMID: 31171907Free PMC Article
Correlations between BCL6 rearrangement and outcome in patients with diffuse large B-cell lymphoma treated with CHOP or R-CHOP.
Shustik J, Han G, Farinha P, Johnson NA, Ben Neriah S, Connors JM, Sehn LH, Horsman DE, Gascoyne RD, Steidl C
Haematologica 2010 Jan;95(1):96-101. Epub 2009 Oct 1 doi: 10.3324/haematol.2009.007203. PMID: 19797725Free PMC Article
BCL6 gene amplification/3q27 gain is associated with unique clinicopathological characteristics among follicular lymphoma without BCL2 gene translocation.
Karube K, Ying G, Tagawa H, Niino D, Aoki R, Kimura Y, Hashikawa K, Suefuji N, Sugita Y, Nomura Y, Shimizu K, Yoshida S, Seto M, Ohshima K
Mod Pathol 2008 Aug;21(8):973-8. Epub 2008 May 23 doi: 10.1038/modpathol.2008.75. PMID: 18500267
BCL6 gene rearrangement and other cytogenetic abnormalities in diffuse large cell lymphoma.
Offit K, Louie DC, Parsa NZ, Roy P, Leung D, Lo Coco F, Zelenetz A, Dalla-Favera R, Chaganti RS
Leuk Lymphoma 1995 Dec;20(1-2):85-9. doi: 10.3109/10428199509054757. PMID: 8750627

Diagnosis

Clinical Significance of BCL2, C-MYC, and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC/BCL2 Proteins and Co-expression of MYC/BCL2/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients.
Ting CY, Chang KM, Kuan JW, Sathar J, Chew LP, Wong OJ, Yusuf Y, Wong L, Samsudin AT, Pana MNBM, Lee SK, Gopal NSR, Puri R, Ong TC, Bahari SK, Goh AS, Teoh CS
Int J Med Sci 2019;16(4):556-566. Epub 2019 Apr 20 doi: 10.7150/ijms.27610. PMID: 31171907Free PMC Article
Most primary adrenal lymphomas are diffuse large B-cell lymphomas with non-germinal center B-cell phenotype, BCL6 gene rearrangement and poor prognosis.
Mozos A, Ye H, Chuang WY, Chu JS, Huang WT, Chen HK, Hsu YH, Bacon CM, Du MQ, Campo E, Chuang SS
Mod Pathol 2009 Sep;22(9):1210-7. Epub 2009 Jun 12 doi: 10.1038/modpathol.2009.87. PMID: 19525926
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 doi: 10.1182/blood-2002-08-2482. PMID: 12738680
Rearrangement of the BCL6 gene in B-cell lymphoid neoplasms: comparison with lymphomas associated with BCL2 rearrangement.
Muramatsu M, Akasaka T, Kadowaki N, Ohno H, Yamabe H, Edamura S, Dor S, Mori T, Okuma M, Fukuhara S
Br J Haematol 1996 Jun;93(4):911-20. doi: 10.1046/j.1365-2141.1996.d01-1728.x. PMID: 8703825
BCL6 gene rearrangement and other cytogenetic abnormalities in diffuse large cell lymphoma.
Offit K, Louie DC, Parsa NZ, Roy P, Leung D, Lo Coco F, Zelenetz A, Dalla-Favera R, Chaganti RS
Leuk Lymphoma 1995 Dec;20(1-2):85-9. doi: 10.3109/10428199509054757. PMID: 8750627

Therapy

Clinical Significance of BCL2, C-MYC, and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC/BCL2 Proteins and Co-expression of MYC/BCL2/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients.
Ting CY, Chang KM, Kuan JW, Sathar J, Chew LP, Wong OJ, Yusuf Y, Wong L, Samsudin AT, Pana MNBM, Lee SK, Gopal NSR, Puri R, Ong TC, Bahari SK, Goh AS, Teoh CS
Int J Med Sci 2019;16(4):556-566. Epub 2019 Apr 20 doi: 10.7150/ijms.27610. PMID: 31171907Free PMC Article
Cytogenetic abnormalities precede morphological abnormalities in developing malignant conditions: report of 2 cases.
Symons WA, Flynt FL, Mendiola C, Ortega V, Higgins RA, Velagaleti GV
Exp Mol Pathol 2013 Feb;94(1):98-102. Epub 2012 Oct 10 doi: 10.1016/j.yexmp.2012.10.003. PMID: 23064050
Correlations between BCL6 rearrangement and outcome in patients with diffuse large B-cell lymphoma treated with CHOP or R-CHOP.
Shustik J, Han G, Farinha P, Johnson NA, Ben Neriah S, Connors JM, Sehn LH, Horsman DE, Gascoyne RD, Steidl C
Haematologica 2010 Jan;95(1):96-101. Epub 2009 Oct 1 doi: 10.3324/haematol.2009.007203. PMID: 19797725Free PMC Article

Prognosis

Clinical Significance of BCL2, C-MYC, and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC/BCL2 Proteins and Co-expression of MYC/BCL2/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients.
Ting CY, Chang KM, Kuan JW, Sathar J, Chew LP, Wong OJ, Yusuf Y, Wong L, Samsudin AT, Pana MNBM, Lee SK, Gopal NSR, Puri R, Ong TC, Bahari SK, Goh AS, Teoh CS
Int J Med Sci 2019;16(4):556-566. Epub 2019 Apr 20 doi: 10.7150/ijms.27610. PMID: 31171907Free PMC Article
Correlations between BCL6 rearrangement and outcome in patients with diffuse large B-cell lymphoma treated with CHOP or R-CHOP.
Shustik J, Han G, Farinha P, Johnson NA, Ben Neriah S, Connors JM, Sehn LH, Horsman DE, Gascoyne RD, Steidl C
Haematologica 2010 Jan;95(1):96-101. Epub 2009 Oct 1 doi: 10.3324/haematol.2009.007203. PMID: 19797725Free PMC Article
Most primary adrenal lymphomas are diffuse large B-cell lymphomas with non-germinal center B-cell phenotype, BCL6 gene rearrangement and poor prognosis.
Mozos A, Ye H, Chuang WY, Chu JS, Huang WT, Chen HK, Hsu YH, Bacon CM, Du MQ, Campo E, Chuang SS
Mod Pathol 2009 Sep;22(9):1210-7. Epub 2009 Jun 12 doi: 10.1038/modpathol.2009.87. PMID: 19525926
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 doi: 10.1182/blood-2002-08-2482. PMID: 12738680
BCL6 gene rearrangement and other cytogenetic abnormalities in diffuse large cell lymphoma.
Offit K, Louie DC, Parsa NZ, Roy P, Leung D, Lo Coco F, Zelenetz A, Dalla-Favera R, Chaganti RS
Leuk Lymphoma 1995 Dec;20(1-2):85-9. doi: 10.3109/10428199509054757. PMID: 8750627

Clinical prediction guides

Characterisation of immunogenotypes of diffuse large B-cell lymphoma.
Phang KC, Hussin NH, Abdul Rahman F, Tizen NMS, Mansoor A, Masir N
Malays J Pathol 2019 Aug;41(2):101-124. PMID: 31427546
Clinical Significance of BCL2, C-MYC, and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC/BCL2 Proteins and Co-expression of MYC/BCL2/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients.
Ting CY, Chang KM, Kuan JW, Sathar J, Chew LP, Wong OJ, Yusuf Y, Wong L, Samsudin AT, Pana MNBM, Lee SK, Gopal NSR, Puri R, Ong TC, Bahari SK, Goh AS, Teoh CS
Int J Med Sci 2019;16(4):556-566. Epub 2019 Apr 20 doi: 10.7150/ijms.27610. PMID: 31171907Free PMC Article
Correlations between BCL6 rearrangement and outcome in patients with diffuse large B-cell lymphoma treated with CHOP or R-CHOP.
Shustik J, Han G, Farinha P, Johnson NA, Ben Neriah S, Connors JM, Sehn LH, Horsman DE, Gascoyne RD, Steidl C
Haematologica 2010 Jan;95(1):96-101. Epub 2009 Oct 1 doi: 10.3324/haematol.2009.007203. PMID: 19797725Free PMC Article
Rearrangement of the BCL6 gene in B-cell lymphoid neoplasms: comparison with lymphomas associated with BCL2 rearrangement.
Muramatsu M, Akasaka T, Kadowaki N, Ohno H, Yamabe H, Edamura S, Dor S, Mori T, Okuma M, Fukuhara S
Br J Haematol 1996 Jun;93(4):911-20. doi: 10.1046/j.1365-2141.1996.d01-1728.x. PMID: 8703825
BCL6 gene rearrangement and other cytogenetic abnormalities in diffuse large cell lymphoma.
Offit K, Louie DC, Parsa NZ, Roy P, Leung D, Lo Coco F, Zelenetz A, Dalla-Favera R, Chaganti RS
Leuk Lymphoma 1995 Dec;20(1-2):85-9. doi: 10.3109/10428199509054757. PMID: 8750627

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