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

MedGen UID:
1648864
•Concept ID:
C4726596
•
Neoplastic Process
Synonyms: Recurrent Double-Hit Lymphoma; Recurrent High-Grade B-Cell Lymphoma with MYC and BCL2 or BCL6 Rearrangements

Definition

The reemergence of high-grade B-cell lymphoma with MYC and BCL2 or BCL6 rearrangements after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent High Grade B-Cell Lymphoma with MYC and BCL2 or BCL6 Rearrangements

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
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

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
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
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free 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

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
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
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free 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

Therapy

Combination Targeted Therapy in Relapsed Diffuse Large B-Cell Lymphoma.
Melani C, Lakhotia R, Pittaluga S, Phelan JD, Huang DW, Wright G, Simard J, Muppidi J, Thomas CJ, Ceribelli M, Tosto FA, Yang Y, Xu W, Davies-Hill T, Pack SD, Peer CJ, Arisa O, Mena E, Lindenberg L, Bergvall E, Portell CA, Farah RJ, Lee ST, Pradhan A, Morrison C, Tadese A, Juanitez AM, Lu C, Jacob A, Simmons H, Figg WD, Steinberg SM, Jaffe ES, Roschewski M, Staudt LM, Wilson WH
N Engl J Med 2024 Jun 20;390(23):2143-2155. doi: 10.1056/NEJMoa2401532. PMID: 38899693Free PMC Article
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
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
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
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

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
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
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
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free 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

Combination Targeted Therapy in Relapsed Diffuse Large B-Cell Lymphoma.
Melani C, Lakhotia R, Pittaluga S, Phelan JD, Huang DW, Wright G, Simard J, Muppidi J, Thomas CJ, Ceribelli M, Tosto FA, Yang Y, Xu W, Davies-Hill T, Pack SD, Peer CJ, Arisa O, Mena E, Lindenberg L, Bergvall E, Portell CA, Farah RJ, Lee ST, Pradhan A, Morrison C, Tadese A, Juanitez AM, Lu C, Jacob A, Simmons H, Figg WD, Steinberg SM, Jaffe ES, Roschewski M, Staudt LM, Wilson WH
N Engl J Med 2024 Jun 20;390(23):2143-2155. doi: 10.1056/NEJMoa2401532. PMID: 38899693Free PMC Article
Motive and opportunity: MYC rearrangements in high-grade B-cell lymphoma with MYC and BCL2 rearrangements (an LLMPP study).
Hilton LK, Collinge B, Ben-Neriah S, Alduaij W, Shaalan H, Weng AP, Cruz M, Slack GW, Farinha P, Miyata-Takata T, Boyle M, Meissner B, Cook JR, Ondrejka SL, Ott G, Rosenwald A, Campo E, Amador C, Greiner TC, Raess PW, Song JY, Inghirami G, Jaffe ES, Weisenburger DD, Chan WC, Beiske K, Fu K, Delabie J, Pittaluga S, Iqbal J, Wright G, Sehn LH, Savage KJ, Mungall AJ, Feldman AL, Staudt LM, Steidl C, Rimsza LM, Morin RD, Scott DW
Blood 2024 Aug 1;144(5):525-540. doi: 10.1182/blood.2024024251. PMID: 38701426Free 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
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free 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

Recent systematic reviews

Does cell-of-origin or MYC, BCL2 or BCL6 translocation status provide prognostic information beyond the International Prognostic Index score in patients with diffuse large B-cell lymphoma treated with rituximab and chemotherapy? A systematic review.
Schmidt-Hansen M, Berendse S, Marafioti T, McNamara C
Leuk Lymphoma 2017 Oct;58(10):2403-2418. Epub 2017 Feb 9 doi: 10.1080/10428194.2017.1287364. PMID: 28278726
Front-line, dose-escalated immunochemotherapy is associated with a significant progression-free survival advantage in patients with double-hit lymphomas: a systematic review and meta-analysis.
Howlett C, Snedecor SJ, Landsburg DJ, Svoboda J, Chong EA, Schuster SJ, Nasta SD, Feldman T, Rago A, Walsh KM, Weber S, Goy A, Mato A
Br J Haematol 2015 Aug;170(4):504-14. Epub 2015 Apr 24 doi: 10.1111/bjh.13463. PMID: 25907897

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