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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
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

Shadman M
JAMA 2023 Mar 21;329(11):918-932. doi: 10.1001/jama.2023.1946. PMID: 36943212
Poletto S, Novo M, Paruzzo L, Frascione PMM, Vitolo U
Cancer Treat Rev 2022 Nov;110:102443. Epub 2022 Jul 31 doi: 10.1016/j.ctrv.2022.102443. PMID: 35933930
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

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
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
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
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
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

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
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
Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
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
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

Hallek M, Al-Sawaf O
Am J Hematol 2021 Dec 1;96(12):1679-1705. doi: 10.1002/ajh.26367. PMID: 34625994
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
Freedman A, Jacobsen E
Am J Hematol 2020 Mar;95(3):316-327. Epub 2019 Dec 22 doi: 10.1002/ajh.25696. PMID: 31814159
Riedell PA, Smith SM
Cancer 2018 Dec 15;124(24):4622-4632. Epub 2018 Sep 25 doi: 10.1002/cncr.31646. PMID: 30252929
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

Olszewski AJ, Kurt H, Evens AM
Blood 2022 Sep 1;140(9):943-954. doi: 10.1182/blood.2020008374. PMID: 34525177
Jain P, Wang M
Am J Hematol 2019 Jun;94(6):710-725. Epub 2019 Apr 19 doi: 10.1002/ajh.25487. PMID: 30963600
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
Riedell PA, Smith SM
Cancer 2018 Dec 15;124(24):4622-4632. Epub 2018 Sep 25 doi: 10.1002/cncr.31646. PMID: 30252929
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

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
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
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
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
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

Recent systematic reviews

Rizzuto A, Pirrera A, Gigliotta E, Mancuso S, Vullo C, Camarda GM, Rotolo C, Roppolo A, Spoto C, Gentile M, Botta C, Siragusa S
Int J Mol Sci 2023 Jun 9;24(12) doi: 10.3390/ijms24129930. PMID: 37373078Free PMC Article
Aiman W, Ali MA, Basit MA, Omar Z, Suleman M, Hassan M, Jamil T, Anwar MS, Shafique Z, Dhanesar G, Faisal MS, Akerman MJ, Maroules M, Anwer F
Leuk Res 2023 Jun;129:107077. Epub 2023 Apr 14 doi: 10.1016/j.leukres.2023.107077. PMID: 37100025
He W, He F, Hu H
Ann Med 2023 Dec;55(1):1029-1036. doi: 10.1080/07853890.2023.2186480. PMID: 36911885Free PMC Article
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
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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