U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

MedGen UID:
1617477
•Concept ID:
C4524190
•
Neoplastic Process
Synonyms: HGBL with MYC and BCL2 and/or BCL6 Rearrangements; High grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
SNOMED CT: High grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (786909001); High grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (1172704005)

Definition

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

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

Recent clinical studies

Etiology

Double-hit lymphoma: optimizing therapy.
Dunleavy K
Hematology Am Soc Hematol Educ Program 2021 Dec 10;2021(1):157-163. doi: 10.1182/hematology.2021000247. PMID: 34889402Free 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
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
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
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

Double-hit lymphoma: optimizing therapy.
Dunleavy K
Hematology Am Soc Hematol Educ Program 2021 Dec 10;2021(1):157-163. doi: 10.1182/hematology.2021000247. PMID: 34889402Free 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
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
ABC, GCB, and Double-Hit Diffuse Large B-Cell Lymphoma: Does Subtype Make a Difference in Therapy Selection?
Nowakowski GS, Czuczman MS
Am Soc Clin Oncol Educ Book 2015:e449-57. doi: 10.14694/EdBook_AM.2015.35.e449. PMID: 25993209

Prognosis

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
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
ABC, GCB, and Double-Hit Diffuse Large B-Cell Lymphoma: Does Subtype Make a Difference in Therapy Selection?
Nowakowski GS, Czuczman MS
Am Soc Clin Oncol Educ Book 2015:e449-57. doi: 10.14694/EdBook_AM.2015.35.e449. PMID: 25993209

Clinical prediction guides

Dose-adjusted EPOCH and rituximab for the treatment of double expressor and double-hit diffuse large B-cell lymphoma: impact of TP53 mutations on clinical outcome.
Dodero A, Guidetti A, Marino F, Tucci A, Barretta F, Re A, Balzarotti M, Carniti C, Monfrini C, Chiappella A, Cabras A, Facchetti F, Pennisi M, Rahal D, Monti V, Devizzi L, Miceli R, Cocito F, Farina L, Ricci F, Rossi G, Carlo-Stella C, Corradini P
Haematologica 2022 May 1;107(5):1153-1162. doi: 10.3324/haematol.2021.278638. PMID: 34289655Free PMC Article
Pseudolymphomatous Atypical Fibroxanthoma.
Garcia Castro R, Viñolas-Cuadros A, Moyano-Bueno D, Conde-Ferreiros A, Cardeñoso E, Santos-Briz A
Am J Dermatopathol 2020 Dec;42(12):972-976. doi: 10.1097/DAD.0000000000001761. PMID: 32769551
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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...