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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
Jacobsen E
Am J Hematol 2022 Dec;97(12):1638-1651. Epub 2022 Oct 18 doi: 10.1002/ajh.26737. PMID: 36255040
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

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
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
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
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
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

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
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
Riedell PA, Smith SM
Cancer 2018 Dec 15;124(24):4622-4632. Epub 2018 Sep 25 doi: 10.1002/cncr.31646. PMID: 30252929

Prognosis

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

Recent systematic reviews

Travaglino A, Raffone A, Raimondo D, Gencarelli A, Esposito I, Gallo C, Improda FP, Vitale SG, Mollo A, Casadio P, Seracchioli R, Zullo F, Insabato L
Arch Gynecol Obstet 2023 Feb;307(2):379-386. Epub 2022 Mar 28 doi: 10.1007/s00404-022-06531-2. PMID: 35344084Free PMC Article
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
Deng H, Yue JK, Zusman BE, Nwachuku EL, Abou-Al-Shaar H, Upadhyayula PS, Okonkwo DO, Puccio AM
Medicina (Kaunas) 2020 Jun 18;56(6) doi: 10.3390/medicina56060300. PMID: 32570722Free 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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