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Refractory High Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

MedGen UID:
1700179
•Concept ID:
C5206566
•
Neoplastic Process
Synonym: Refractory High-Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

Definition

High-grade B-cell lymphoma with MYC, BCL2, and BCL6 rearrangements that is resistant to treatment. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Molecularly Stratified Treatment Options in Primary Refractory DLBCL/HGBL with MYC and BCL2 or BCL6 Rearrangements (HGBL, NOS with MYC/BCL6).
Witte HM, Riedl J, Künstner A, Fähnrich A, Ketzer J, Fliedner SMJ, Reimer N, Bernard V, von Bubnoff N, Merz H, Busch H, Feller A, Gebauer N
Target Oncol 2023 Sep;18(5):749-765. Epub 2023 Jul 24 doi: 10.1007/s11523-023-00983-5. PMID: 37488307Free PMC Article
ABCs of ADCs in management of relapsed/refractory diffuse large B-cell lymphoma.
Alderuccio JP, Sharman JP
Blood Rev 2022 Nov;56:100967. Epub 2022 Apr 22 doi: 10.1016/j.blre.2022.100967. PMID: 35489963

Recent clinical studies

Etiology

High-grade B-cell lymphoma with a quadruple-hit genetic profile including concurrent MYC, BCL2, BCL6, and CCND1 gene rearrangements.
Gagnon MF, Meyer RG, Weaver EJ, Wood AJ, Dupuy DA Jr, Menachery SJ, Shi M, Baughn LB, Ketterling RP, Peterson JF
Lab Med 2024 Sep 4;55(5):649-654. doi: 10.1093/labmed/lmae017. PMID: 38522075
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
Impact of Double- or Triple-Hit Pathology on Rates and Durability of Radiation Therapy Response Among Patients With Relapsed or Refractory Large B-Cell Lymphoma.
Kim A, Stevenson P, Cassaday RD, Soma L, Fromm JR, Gopal AK, Smith SD, Till B, Lynch RC, Ujjani C, Shadman M, Warren EH, Menon M, Russell K, Tseng YD
Pract Radiat Oncol 2020 Jan-Feb;10(1):44-52. Epub 2019 Oct 1 doi: 10.1016/j.prro.2019.09.013. PMID: 31585205
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

High-grade B-cell lymphoma with a quadruple-hit genetic profile including concurrent MYC, BCL2, BCL6, and CCND1 gene rearrangements.
Gagnon MF, Meyer RG, Weaver EJ, Wood AJ, Dupuy DA Jr, Menachery SJ, Shi M, Baughn LB, Ketterling RP, Peterson JF
Lab Med 2024 Sep 4;55(5):649-654. doi: 10.1093/labmed/lmae017. PMID: 38522075
DA-EPOCH-R therapy for high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements in a patient with renal dysfunction.
Mitobe M, Kawamoto K, Suzuki T, Suwabe T, Shibasaki Y, Masuko M, Inoue K, Miyoshi H, Ohshima K, Sone H, Takizawa J
J Clin Exp Hematop 2021 Mar 18;61(1):42-47. Epub 2021 Feb 6 doi: 10.3960/jslrt.20043. PMID: 33551436Free PMC Article
Characterization of two new high-grade B-cell lymphoma cell lines with MYC and BCL2 rearrangements that are suitable for in vitro drug sensitivity studies.
Dheur MS, Poirel HA, Ameye G, Tilman G, Saussoy P, Defour JP, Camboni A, Van Den Neste E, Coulie PG, van Baren N
Leuk Lymphoma 2019 Apr;60(4):1043-1052. Epub 2018 Oct 2 doi: 10.1080/10428194.2018.1508663. PMID: 30277098
Advances in pathological understanding of high-grade B cell lymphomas.
Li S, Lin P, Medeiros LJ
Expert Rev Hematol 2018 Aug;11(8):637-648. Epub 2018 Jul 10 doi: 10.1080/17474086.2018.1494567. PMID: 29989509

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
ABCs of ADCs in management of relapsed/refractory diffuse large B-cell lymphoma.
Alderuccio JP, Sharman JP
Blood Rev 2022 Nov;56:100967. Epub 2022 Apr 22 doi: 10.1016/j.blre.2022.100967. PMID: 35489963
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
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

Impact of Double- or Triple-Hit Pathology on Rates and Durability of Radiation Therapy Response Among Patients With Relapsed or Refractory Large B-Cell Lymphoma.
Kim A, Stevenson P, Cassaday RD, Soma L, Fromm JR, Gopal AK, Smith SD, Till B, Lynch RC, Ujjani C, Shadman M, Warren EH, Menon M, Russell K, Tseng YD
Pract Radiat Oncol 2020 Jan-Feb;10(1):44-52. Epub 2019 Oct 1 doi: 10.1016/j.prro.2019.09.013. PMID: 31585205
Characterization of two new high-grade B-cell lymphoma cell lines with MYC and BCL2 rearrangements that are suitable for in vitro drug sensitivity studies.
Dheur MS, Poirel HA, Ameye G, Tilman G, Saussoy P, Defour JP, Camboni A, Van Den Neste E, Coulie PG, van Baren N
Leuk Lymphoma 2019 Apr;60(4):1043-1052. Epub 2018 Oct 2 doi: 10.1080/10428194.2018.1508663. PMID: 30277098
Challenges and Opportunities for High-grade B-Cell Lymphoma With MYC and BCL2 and/or BCL6 Rearrangement (Double-hit Lymphoma).
Zeng D, Desai A, Yan F, Gong T, Ye H, Ahmed M, Nomie K, Romaguera J, Champlin R, Li S, Wang M
Am J Clin Oncol 2019 Mar;42(3):304-316. doi: 10.1097/COC.0000000000000427. PMID: 29419530
Advances in pathological understanding of high-grade B cell lymphomas.
Li S, Lin P, Medeiros LJ
Expert Rev Hematol 2018 Aug;11(8):637-648. Epub 2018 Jul 10 doi: 10.1080/17474086.2018.1494567. PMID: 29989509
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
Impact of Double- or Triple-Hit Pathology on Rates and Durability of Radiation Therapy Response Among Patients With Relapsed or Refractory Large B-Cell Lymphoma.
Kim A, Stevenson P, Cassaday RD, Soma L, Fromm JR, Gopal AK, Smith SD, Till B, Lynch RC, Ujjani C, Shadman M, Warren EH, Menon M, Russell K, Tseng YD
Pract Radiat Oncol 2020 Jan-Feb;10(1):44-52. Epub 2019 Oct 1 doi: 10.1016/j.prro.2019.09.013. PMID: 31585205

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