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High grade B-cell lymphoma with MYC and/ or BCL2 and/or BCL6 rearrangement

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; High grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements; high grade B-cell lymphoma with MYC and/ or BCL2 and/or BCL6 rearrangement; 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)
 
Monarch Initiative: MONDO:0018813
Orphanet: ORPHA480541

Definition

A rare aggressive B-cell non-Hodgkin lymphoma with characteristics of rearrangement in MYC and BCL2 and/or BCL6 (so-called double-hit or triple-hit lymphoma). The category includes double-hit cases with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, blastoid cases with a double-hit, and cases with a DLBCL not otherwise specified morphology with a double-hit. It refers only to de novo cases, not to lymphomas with a history of pre-existing or coexistent indolent lymphoma. Patients typically present with widespread disease, including involvement of lymph nodes, bone marrow and central nervous system. [from SNOMEDCT_US]

Professional guidelines

PubMed

Diagnosis and Treatment of Chronic Lymphocytic Leukemia: A Review.
Shadman M
JAMA 2023 Mar 21;329(11):918-932. doi: 10.1001/jama.2023.1946. PMID: 36943212
Follicular lymphoma: 2023 update on diagnosis and management.
Jacobsen E
Am J Hematol 2022 Dec;97(12):1638-1651. Epub 2022 Oct 18 doi: 10.1002/ajh.26737. PMID: 36255040
Diagnosis and management of AML in adults: 2022 recommendations from an international expert panel on behalf of the ELN.
Döhner H, Wei AH, Appelbaum FR, Craddock C, DiNardo CD, Dombret H, Ebert BL, Fenaux P, Godley LA, Hasserjian RP, Larson RA, Levine RL, Miyazaki Y, Niederwieser D, Ossenkoppele G, Röllig C, Sierra J, Stein EM, Tallman MS, Tien HF, Wang J, Wierzbowska A, Löwenberg B
Blood 2022 Sep 22;140(12):1345-1377. doi: 10.1182/blood.2022016867. PMID: 35797463

Recent clinical studies

Etiology

Molecular determinants of clinical outcomes in a real-world diffuse large B-cell lymphoma population.
Alduaij W, Collinge B, Ben-Neriah S, Jiang A, Hilton LK, Boyle M, Meissner B, Chong L, Miyata-Takata T, Slack GW, Farinha P, Craig JW, Lytle A, Savage KJ, Villa D, Gerrie AS, Freeman CL, Gascoyne RD, Connors JM, Morin RD, Sehn LH, Mungall AJ, Steidl C, Scott DW
Blood 2023 May 18;141(20):2493-2507. doi: 10.1182/blood.2022018248. PMID: 36302166
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
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
Defining and treating high-grade B-cell lymphoma, NOS.
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
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

Therapy

Chronic lymphocytic leukemia: 2022 update on diagnostic and therapeutic procedures.
Hallek M, Al-Sawaf O
Am J Hematol 2021 Dec 1;96(12):1679-1705. doi: 10.1002/ajh.26367. PMID: 34625994
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
Follicular lymphoma: 2020 update on diagnosis and management.
Freedman A, Jacobsen E
Am J Hematol 2020 Mar;95(3):316-327. Epub 2019 Dec 22 doi: 10.1002/ajh.25696. PMID: 31814159
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

Prognosis

Defining and treating high-grade B-cell lymphoma, NOS.
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
Venetoclax combined with decitabine or azacitidine in treatment-naive, elderly patients with acute myeloid leukemia.
DiNardo CD, Pratz K, Pullarkat V, Jonas BA, Arellano M, Becker PS, Frankfurt O, Konopleva M, Wei AH, Kantarjian HM, Xu T, Hong WJ, Chyla B, Potluri J, Pollyea DA, Letai A
Blood 2019 Jan 3;133(1):7-17. Epub 2018 Oct 25 doi: 10.1182/blood-2018-08-868752. PMID: 30361262Free 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
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
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
Prognostic Significance of MYC Rearrangement and Translocation Partner in Diffuse Large B-Cell Lymphoma: A Study by the Lunenburg Lymphoma Biomarker Consortium.
Rosenwald A, Bens S, Advani R, Barrans S, Copie-Bergman C, Elsensohn MH, Natkunam Y, Calaminici M, Sander B, Baia M, Smith A, Painter D, Pham L, Zhao S, Ziepert M, Jordanova ES, Molina TJ, Kersten MJ, Kimby E, Klapper W, Raemaekers J, Schmitz N, Jardin F, Stevens WBC, Hoster E, Hagenbeek A, Gribben JG, Siebert R, Gascoyne RD, Scott DW, Gaulard P, Salles G, Burton C, de Jong D, Sehn LH, Maucort-Boulch D
J Clin Oncol 2019 Dec 10;37(35):3359-3368. Epub 2019 Sep 9 doi: 10.1200/JCO.19.00743. PMID: 31498031
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

Recent systematic reviews

Molecular-Biology-Driven Frontline Treatment for Chronic Lymphocytic Leukemia: A Network Meta-Analysis of Randomized Clinical Trials.
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Arch Gynecol Obstet 2023 Feb;307(2):379-386. Epub 2022 Mar 28 doi: 10.1007/s00404-022-06531-2. PMID: 35344084Free PMC Article
Prognostic value of Bcl2 and Bcl6 in primary cutaneous large B-cell lymphoma: A systematic review and meta-analysis.
Russo D, Travaglino A, Varricchio S, Pace M, Cretella P, Baldo A, Severino A, Picardi M, Mascolo M
Pathol Res Pract 2022 Apr;232:153812. Epub 2022 Feb 22 doi: 10.1016/j.prp.2022.153812. PMID: 35220171
A systematic review of p53 regulation of oxidative stress in skeletal muscle.
Beyfuss K, Hood DA
Redox Rep 2018 Dec;23(1):100-117. Epub 2018 Jan 3 doi: 10.1080/13510002.2017.1416773. PMID: 29298131Free PMC Article
Characteristics and prognosis of glomangiopericytomas: A systematic review.
Park ES, Kim J, Jun SY
Head Neck 2017 Sep;39(9):1897-1909. Epub 2017 May 12 doi: 10.1002/hed.24818. PMID: 28497509

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