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High Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

MedGen UID:
1388063
•Concept ID:
C4330265
•
Neoplastic Process
Synonyms: High-Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements; Triple Hit Lymphoma; Triple-Hit Lymphoma

Definition

A rare B-cell non-Hodgkin lymphoma that is characterized by the abnormal rearrangement of MYC gene, BCL2 gene, and BCL6 gene. Patients with this type of lymphoma usually respond poorly to standard treatments and have a poor prognosis. [from NCI]

Professional guidelines

PubMed

Current Treatment of Burkitt Lymphoma and High-Grade B-Cell Lymphomas.
Johnson PC, Abramson JS
Oncology (Williston Park) 2022 Aug 10;36(8):499-505. doi: 10.46883/2022.25920970. PMID: 36001789
Perspectives on chemotherapy for the management of double-hit lymphoma.
Al-Juhaishi T, Mckay J, Sindel A, Yazbeck V
Expert Opin Pharmacother 2020 Apr;21(6):653-661. Epub 2020 Feb 17 doi: 10.1080/14656566.2020.1727445. PMID: 32066288
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

Lisocabtagene maraleucel for patients with relapsed or refractory large B-cell lymphomas (TRANSCEND NHL 001): a multicentre seamless design study.
Abramson JS, Palomba ML, Gordon LI, Lunning MA, Wang M, Arnason J, Mehta A, Purev E, Maloney DG, Andreadis C, Sehgal A, Solomon SR, Ghosh N, Albertson TM, Garcia J, Kostic A, Mallaney M, Ogasawara K, Newhall K, Kim Y, Li D, Siddiqi T
Lancet 2020 Sep 19;396(10254):839-852. Epub 2020 Sep 1 doi: 10.1016/S0140-6736(20)31366-0. PMID: 32888407
Molecular High-Grade B-Cell Lymphoma: Defining a Poor-Risk Group That Requires Different Approaches to Therapy.
Sha C, Barrans S, Cucco F, Bentley MA, Care MA, Cummin T, Kennedy H, Thompson JS, Uddin R, Worrillow L, Chalkley R, van Hoppe M, Ahmed S, Maishman T, Caddy J, Schuh A, Mamot C, Burton C, Tooze R, Davies A, Du MQ, Johnson PWM, Westhead DR
J Clin Oncol 2019 Jan 20;37(3):202-212. Epub 2018 Dec 3 doi: 10.1200/JCO.18.01314. PMID: 30523719Free PMC Article
Double-Hit Gene Expression Signature Defines a Distinct Subgroup of Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma.
Ennishi D, Jiang A, Boyle M, Collinge B, Grande BM, Ben-Neriah S, Rushton C, Tang J, Thomas N, Slack GW, Farinha P, Takata K, Miyata-Takata T, Craig J, Mottok A, Meissner B, Saberi S, Bashashati A, Villa D, Savage KJ, Sehn LH, Kridel R, Mungall AJ, Marra MA, Shah SP, Steidl C, Connors JM, Gascoyne RD, Morin RD, Scott DW
J Clin Oncol 2019 Jan 20;37(3):190-201. Epub 2018 Dec 3 doi: 10.1200/JCO.18.01583. PMID: 30523716Free PMC Article
Dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab) in untreated aggressive diffuse large B-cell lymphoma with MYC rearrangement: a prospective, multicentre, single-arm phase 2 study.
Dunleavy K, Fanale MA, Abramson JS, Noy A, Caimi PF, Pittaluga S, Parekh S, Lacasce A, Hayslip JW, Jagadeesh D, Nagpal S, Lechowicz MJ, Gaur R, Lucas A, Melani C, Roschewski M, Steinberg SM, Jaffe ES, Kahl B, Friedberg JW, Little RF, Bartlett NL, Wilson WH
Lancet Haematol 2018 Dec;5(12):e609-e617. doi: 10.1016/S2352-3026(18)30177-7. PMID: 30501868Free 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

Diagnosis

High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Diagnostic approaches and future directions in Burkitt lymphoma and high-grade B-cell lymphoma.
King RL, Hsi ED, Chan WC, Piris MA, Cook JR, Scott DW, Swerdlow SH
Virchows Arch 2023 Jan;482(1):193-205. Epub 2022 Sep 3 doi: 10.1007/s00428-022-03404-6. PMID: 36057749
High-grade B-cell lymphoma: how to diagnose and treat.
Novo M, Castellino A, Nicolosi M, Santambrogio E, Vassallo F, Chiappella A, Vitolo U
Expert Rev Hematol 2019 Jul;12(7):497-506. Epub 2019 Jun 19 doi: 10.1080/17474086.2019.1624157. PMID: 31150587
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements with diffuse large B-cell lymphoma morphology.
Scott DW, King RL, Staiger AM, Ben-Neriah S, Jiang A, Horn H, Mottok A, Farinha P, Slack GW, Ennishi D, Schmitz N, Pfreundschuh M, Nowakowski GS, Kahl BS, Connors JM, Gascoyne RD, Ott G, Macon WR, Rosenwald A
Blood 2018 May 3;131(18):2060-2064. Epub 2018 Feb 23 doi: 10.1182/blood-2017-12-820605. PMID: 29475959Free PMC Article
Diagnosis and classification of hematologic malignancies on the basis of genetics.
Taylor J, Xiao W, Abdel-Wahab O
Blood 2017 Jul 27;130(4):410-423. Epub 2017 Jun 9 doi: 10.1182/blood-2017-02-734541. PMID: 28600336Free PMC Article

Therapy

Lisocabtagene maraleucel for patients with relapsed or refractory large B-cell lymphomas (TRANSCEND NHL 001): a multicentre seamless design study.
Abramson JS, Palomba ML, Gordon LI, Lunning MA, Wang M, Arnason J, Mehta A, Purev E, Maloney DG, Andreadis C, Sehgal A, Solomon SR, Ghosh N, Albertson TM, Garcia J, Kostic A, Mallaney M, Ogasawara K, Newhall K, Kim Y, Li D, Siddiqi T
Lancet 2020 Sep 19;396(10254):839-852. Epub 2020 Sep 1 doi: 10.1016/S0140-6736(20)31366-0. PMID: 32888407
Molecular High-Grade B-Cell Lymphoma: Defining a Poor-Risk Group That Requires Different Approaches to Therapy.
Sha C, Barrans S, Cucco F, Bentley MA, Care MA, Cummin T, Kennedy H, Thompson JS, Uddin R, Worrillow L, Chalkley R, van Hoppe M, Ahmed S, Maishman T, Caddy J, Schuh A, Mamot C, Burton C, Tooze R, Davies A, Du MQ, Johnson PWM, Westhead DR
J Clin Oncol 2019 Jan 20;37(3):202-212. Epub 2018 Dec 3 doi: 10.1200/JCO.18.01314. PMID: 30523719Free PMC Article
Double-Hit Gene Expression Signature Defines a Distinct Subgroup of Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma.
Ennishi D, Jiang A, Boyle M, Collinge B, Grande BM, Ben-Neriah S, Rushton C, Tang J, Thomas N, Slack GW, Farinha P, Takata K, Miyata-Takata T, Craig J, Mottok A, Meissner B, Saberi S, Bashashati A, Villa D, Savage KJ, Sehn LH, Kridel R, Mungall AJ, Marra MA, Shah SP, Steidl C, Connors JM, Gascoyne RD, Morin RD, Scott DW
J Clin Oncol 2019 Jan 20;37(3):190-201. Epub 2018 Dec 3 doi: 10.1200/JCO.18.01583. PMID: 30523716Free PMC Article
Dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab) in untreated aggressive diffuse large B-cell lymphoma with MYC rearrangement: a prospective, multicentre, single-arm phase 2 study.
Dunleavy K, Fanale MA, Abramson JS, Noy A, Caimi PF, Pittaluga S, Parekh S, Lacasce A, Hayslip JW, Jagadeesh D, Nagpal S, Lechowicz MJ, Gaur R, Lucas A, Melani C, Roschewski M, Steinberg SM, Jaffe ES, Kahl B, Friedberg JW, Little RF, Bartlett NL, Wilson WH
Lancet Haematol 2018 Dec;5(12):e609-e617. doi: 10.1016/S2352-3026(18)30177-7. PMID: 30501868Free 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

Prognosis

Double-Hit Gene Expression Signature Defines a Distinct Subgroup of Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma.
Ennishi D, Jiang A, Boyle M, Collinge B, Grande BM, Ben-Neriah S, Rushton C, Tang J, Thomas N, Slack GW, Farinha P, Takata K, Miyata-Takata T, Craig J, Mottok A, Meissner B, Saberi S, Bashashati A, Villa D, Savage KJ, Sehn LH, Kridel R, Mungall AJ, Marra MA, Shah SP, Steidl C, Connors JM, Gascoyne RD, Morin RD, Scott DW
J Clin Oncol 2019 Jan 20;37(3):190-201. Epub 2018 Dec 3 doi: 10.1200/JCO.18.01583. PMID: 30523716Free PMC Article
Dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab) in untreated aggressive diffuse large B-cell lymphoma with MYC rearrangement: a prospective, multicentre, single-arm phase 2 study.
Dunleavy K, Fanale MA, Abramson JS, Noy A, Caimi PF, Pittaluga S, Parekh S, Lacasce A, Hayslip JW, Jagadeesh D, Nagpal S, Lechowicz MJ, Gaur R, Lucas A, Melani C, Roschewski M, Steinberg SM, Jaffe ES, Kahl B, Friedberg JW, Little RF, Bartlett NL, Wilson WH
Lancet Haematol 2018 Dec;5(12):e609-e617. doi: 10.1016/S2352-3026(18)30177-7. PMID: 30501868Free PMC Article
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements with diffuse large B-cell lymphoma morphology.
Scott DW, King RL, Staiger AM, Ben-Neriah S, Jiang A, Horn H, Mottok A, Farinha P, Slack GW, Ennishi D, Schmitz N, Pfreundschuh M, Nowakowski GS, Kahl BS, Connors JM, Gascoyne RD, Ott G, Macon WR, Rosenwald A
Blood 2018 May 3;131(18):2060-2064. Epub 2018 Feb 23 doi: 10.1182/blood-2017-12-820605. PMID: 29475959Free PMC Article
Diagnosis and classification of hematologic malignancies on the basis of genetics.
Taylor J, Xiao W, Abdel-Wahab O
Blood 2017 Jul 27;130(4):410-423. Epub 2017 Jun 9 doi: 10.1182/blood-2017-02-734541. PMID: 28600336Free 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

Clinical prediction guides

High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study.
Zayac AS, Landsburg DJ, Hughes ME, Bock AM, Nowakowski GS, Ayers EC, Girton M, Hu M, Beckman AK, Li S, Medeiros LJ, Chang JE, Stepanovic A, Kurt H, Sandoval-Sus J, Ansari-Lari MA, Kothari SK, Kress A, Xu ML, Torka P, Sundaram S, Smith SD, Naresh KN, Karimi YH, Epperla N, Bond DA, Farooq U, Saad M, Evens AM, Pandya K, Naik SG, Kamdar M, Haverkos B, Karmali R, Oh TS, Vose JM, Nutsch H, Rubinstein PG, Chaudhry A, Olszewski AJ
Blood Adv 2023 Nov 14;7(21):6381-6394. doi: 10.1182/bloodadvances.2023009731. PMID: 37171397Free PMC Article
Mutational landscape of high-grade B-cell lymphoma with MYC-, BCL2 and/or BCL6 rearrangements characterized by whole-exome sequencing.
Künstner A, Witte HM, Riedl J, Bernard V, Stölting S, Merz H, Olschewski V, Peter W, Ketzer J, Busch Y, Trojok P, Bubnoff NV, Busch H, Feller AC, Gebauer N
Haematologica 2022 Aug 1;107(8):1850-1863. doi: 10.3324/haematol.2021.279631. PMID: 34788985Free PMC Article
Dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab) in untreated aggressive diffuse large B-cell lymphoma with MYC rearrangement: a prospective, multicentre, single-arm phase 2 study.
Dunleavy K, Fanale MA, Abramson JS, Noy A, Caimi PF, Pittaluga S, Parekh S, Lacasce A, Hayslip JW, Jagadeesh D, Nagpal S, Lechowicz MJ, Gaur R, Lucas A, Melani C, Roschewski M, Steinberg SM, Jaffe ES, Kahl B, Friedberg JW, Little RF, Bartlett NL, Wilson WH
Lancet Haematol 2018 Dec;5(12):e609-e617. doi: 10.1016/S2352-3026(18)30177-7. PMID: 30501868Free PMC Article
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements with diffuse large B-cell lymphoma morphology.
Scott DW, King RL, Staiger AM, Ben-Neriah S, Jiang A, Horn H, Mottok A, Farinha P, Slack GW, Ennishi D, Schmitz N, Pfreundschuh M, Nowakowski GS, Kahl BS, Connors JM, Gascoyne RD, Ott G, Macon WR, Rosenwald A
Blood 2018 May 3;131(18):2060-2064. Epub 2018 Feb 23 doi: 10.1182/blood-2017-12-820605. PMID: 29475959Free PMC Article
MYC/BCL2 double-hit high-grade B-cell lymphoma.
Li S, Lin P, Young KH, Kanagal-Shamanna R, Yin CC, Medeiros LJ
Adv Anat Pathol 2013 Sep;20(5):315-26. doi: 10.1097/PAP.0b013e3182a289f2. PMID: 23939148

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