Extra copies of MYC, BCL2, and BCL6 and outcome in patients with diffuse large B-cell lymphoma Journal Article


Authors: Sermer, D.; Bobillo, S.; Dogan, A.; Zhang, Y.; Seshan, V.; Lavery, J. A.; Batlevi, C.; Caron, P.; Hamilton, A.; Hamlin, P.; Horwitz, S.; Joffe, E.; Kumar, A.; Matasar, M.; Noy, A.; Owens, C.; Moskowitz, A.; Palomba, M. L.; Straus, D.; von Keudell, G.; Rodriguez-Rivera, I.; Falchi, L.; Zelenetz, A.; Yahalom, J.; Younes, A.
Article Title: Extra copies of MYC, BCL2, and BCL6 and outcome in patients with diffuse large B-cell lymphoma
Abstract: High-grade B-cell lymphoma (HGBL) with translocations involving MYC and BCL2 or BCL6 comprises;10% of cases of diffuse large B-cell lymphoma (DLBCL) and carries a poor prognosis. The incidence, prognosis, and optimal therapy for DLBCL harboring extra copies of the genes MYC, BCL2, and BCL6, rather than their genetic translocations, are unknown. In this retrospective, single-center study we identified 144 DLBCL cases including 46 patients with classic HGBL with double-hit or triple-hit chromosomal translocations (DHL), 55 with extra copies of MYC in addition to aberrations (extra copies or translocations) of BCL2 and/or BCL6 but did not meet the criteria for HGBL (EC group), and 43 without any aberrations of MYC, BCL2, or BCL6 (wild type [WT]). Unfavorable baseline characteristics had similar frequency in the EC and WT groups, but were significantly more prevalent in the DHL group. With a median follow-up of 36 months, the 2-year event-free survival (EFS) was similar between the WT and EC groups at 77% (95% confidence interval [CI], 65-90) and 82% (95% CI, 72-93), respectively. In contrast, the 2-year EFS of the DHL group was 63% (95% CI, 51-79). The 2-year overall survival in the WT, EC, and DHL groups was 86% (95% CI, 76-97), 89% (95% CI, 81-98), and 74% (95% CI, 62-88), respectively. Among patients treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), the EC group had outcomes similar to those of the WT group. Our results indicate that patients with DLBCL with extra gene copies of MYC, BCL2, and BCL6 fare differently from those with HGBL and respond well to standard R-CHOP therapy. © 2020 by The American Society of Hematology
Journal Title: Blood Advances
Volume: 4
Issue: 14
ISSN: 2473-9529
Publisher: American Society of Hematology  
Date Published: 2020-07-28
Start Page: 3382
End Page: 3390
Language: English
DOI: 10.1182/bloodadvances.2020001551
PUBMED: 32722781
PROVIDER: scopus
PMCID: PMC7391142
DOI/URL:
Notes: Article -- Export Date: 1 October 2020 -- Source: Scopus
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  1. Venkatraman Ennapadam Seshan
    382 Seshan
  2. Joachim Yahalom
    625 Yahalom
  3. Ariela Noy
    351 Noy
  4. Maria Lia Palomba
    415 Palomba
  5. Steven M Horwitz
    645 Horwitz
  6. Andrew D Zelenetz
    767 Zelenetz
  7. Alison Moskowitz
    339 Moskowitz
  8. Paul Hamlin
    277 Hamlin
  9. Matthew J Matasar
    289 Matasar
  10. Philip C Caron
    90 Caron
  11. David J Straus
    356 Straus
  12. Anita Kumar
    180 Kumar
  13. Connie Wing-Ching Lee Batlevi
    176 Batlevi
  14. Anas Younes
    319 Younes
  15. Ahmet Dogan
    454 Dogan
  16. Colette Ngozi Owens
    66 Owens
  17. Erel Joffe
    82 Joffe
  18. Yanming Zhang
    199 Zhang
  19. Jessica Ann Lavery
    79 Lavery
  20. David Sermer
    12 Sermer
  21. Lorenzo Falchi
    107 Falchi