Document Type
Article
Journal Title
Blood Advances
Publication Date
2023
Volume
7
Abstract
In this multi-institutional retrospective study, we examined the characteristics and outcomes of 160 patients with high-grade B-cell lymphoma, not otherwise specified (HGBL-NOS)-a rare category defined by high-grade morphologic features and lack of MYC rearrangements with BCL2 and/or BCL6 rearrangements ("double hit"). Our results show that HGBL-NOS tumors are heterogeneous: 83% of patients had a germinal center B-cell immunophenotype, 37% a dual-expressor immunophenotype (MYC and BCL2 expression), 28% MYC rearrangement, 13% BCL2 rearrangement, and 11% BCL6 rearrangement. Most patients presented with stage IV disease, a high serum lactate dehydrogenase, and other high-risk clinical factors. Most frequent first-line regimens included dose-adjusted cyclophosphamide, doxorubicin, vincristine, and etoposide, with rituximab and prednisone (DA-EPOCH-R; 43%); rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP; 33%); or other intensive chemotherapy programs. We found no significant differences in the rates of complete response (CR), progression-free survival (PFS), or overall survival (OS) between these chemotherapy regimens. CR was attained by 69% of patients. PFS at 2 years was 55.2% and OS was 68.1%. In a multivariable model, the main prognostic factors for PFS and OS were poor performance status, lactate dehydrogenase >3 × upper limit of normal, and a dual-expressor immunophenotype. Age >60 years or presence of MYC rearrangement were not prognostic, but patients with TP53 alterations had a dismal PFS. Presence of MYC rearrangement was not predictive of better PFS in patients treated with DA-EPOCH-R vs R-CHOP. Improvements in the diagnostic criteria and therapeutic approaches beyond dose-intense chemotherapy are needed to overcome the unfavorable prognosis of patients with HGBL-NOS.
MeSH Headings
Humans, Middle Aged, Rituximab, Retrospective Studies, Lymphoma, Large B-Cell, Diffuse, Prednisone, Vincristine, Proto-Oncogene Proteins c-bcl-2, Proto-Oncogene Proteins c-myc, Cyclophosphamide, Doxorubicin, Etoposide, Lactate Dehydrogenases
DOI Link
ISSN
2473-9537
Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-No Derivative Works 4.0 International License.
Recommended Citation
Zayac, Adam S.; Landsburg, Daniel J.; Hughes, Mitchell E.; Bock, Allison M.; Nowakowski, Grzegorz S.; Ayers, Emily C.; Girton, Mark; Hu, Marie; Beckman, Amy K.; Li, Shaoying; Medeiros, L Jeffrey; Chang, Julie E.; Stepanovic, Adam; Kurt, Habibe; Sandoval-Sus, Jose; Ansari-Lari, M Ali; Kothari, Shalin K.; Kress, Anna; Xu, Mina L.; Torka, Pallawi; Sundaram, Suchitra; Smith, Stephen D.; Naresh, Kikkeri N.; Karimi, Yasmin H.; Epperla, Narendranath; Bond, David A.; Farooq, Umar; Saad, Mahak; Evens, Andrew M.; Pandya, Karan; Naik, Seema G.; Kamdar, Manali; Haverkos, Bradley; Karmali, Reem; Oh, Timothy S.; Vose, Julie M.; Nutsch, Heather; Rubinstein, Paul G.; Chaudhry, Amina; and Olszewski, Adam J., "High-Grade B-cell Lymphoma, Not Otherwise Specified: A Multi-Institutional Retrospective Study" (2023). Journal Articles: Oncology and Hematology. 12.
https://digitalcommons.unmc.edu/com_onchem_articles/12