| Title |
High-grade B-cell lymphoma, not otherwise specified: a multi-institutional retrospective study
|
|---|---|
| Published in |
Blood Advances, October 2023
|
| DOI | 10.1182/bloodadvances.2023009731 |
| Pubmed ID | |
| Authors |
Adam Stephen Zayac, Daniel J. Landsburg, Mitchell E. Hughes, Allison M. Bock, Grzegorz S. Nowakowski, Emily C. Ayers, Mark Ryan Girton, Marie Hu, Amy K. Beckman, Shaoying Li, L. Jeffrey Medeiros, Julie E Chang, Adam Stepanovic, Habibe Kurt, Jose Sandoval-Sus, Mohammad Ali Ansari-Lari, Shalin K. Kothari, Anna Kress, Mina L Xu, Pallawi Torka, Suchitra Sundaram, Stephen D Smith, Kikkeri N Naresh, Yasmin H. Karimi, Narendranath Epperla, David A Bond, Umar Farooq, Mahak Saad, Andrew M Evens, Karan Pandya, Seema G. Naik, Manali Kamdar, Bradley M Haverkos, Reem Karmali, Timothy S Oh, Julie M Vose, Heather R Nutsch, Paul G. Rubinstein, Amina Chaudhry, Adam J Olszewski |
| Abstract |
In this multi-institutional retrospective study, we examined characteristics and outcomes of 160 patients with high-grade B-cell lymphoma, not otherwise specified (HGBL-NOS). This rare lymphoma category is defined by high-grade morphologic features, most commonly Burkitt-like, and lack of MYC rearrangements with BCL2 and/or BCL6 rearrangements (so-called double-hit). Our results show that HGBL-NOS tumors are heterogeneous: 83% had a germinal center B-cell immunophenotype, 37% a dual expressor immunophenotype (MYC and BCL2 expression), 28% (single-hit) MYC rearrangement, 13% BCL2 rearrangement, and 11% BCL6 rearrangement. Most patients presented with stage 4 disease, a high serum lactate dehydrogenase, and other high-risk clinical factors. Most frequent first-line regimens included DA-EPOCH-R (43%), R-CHOP (33%), or other intensive chemotherapy programs (11%). We found no significant differences in the rates of complete response (CR, P=0.32), progression-free (PFS, P=0.82), or overall survival (OS, P=0.60) between these chemotherapy regimens. CR was attained by 69% of patients. PFS at 2 years was 55.2% (95%CI, 46.9-62.7), and OS was 68.1% (95%CI, 59.7-75.0). In a multivariable model, the main prognostic factors for PFS and OS were poor performance status, lactate dehydrogenase >3x 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 (13% at 2 years). Presence of MYC rearrangement was not predictive of better PFS in patients treated with DA-EPOCH-R versus 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. |
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|---|---|---|
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| Spain | 2 | 8% |
| Ecuador | 1 | 4% |
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| Unknown | 9 | 38% |
Demographic breakdown
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| Science communicators (journalists, bloggers, editors) | 1 | 4% |
Mendeley demographics
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|---|---|---|
| Unknown | 39 | 100% |
Demographic breakdown
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|---|---|---|
| Student > Bachelor | 7 | 18% |
| Other | 6 | 15% |
| Student > Ph. D. Student | 2 | 5% |
| Student > Master | 2 | 5% |
| Student > Doctoral Student | 1 | 3% |
| Other | 4 | 10% |
| Unknown | 17 | 44% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 17 | 44% |
| Biochemistry, Genetics and Molecular Biology | 2 | 5% |
| Agricultural and Biological Sciences | 2 | 5% |
| Arts and Humanities | 1 | 3% |
| Unknown | 17 | 44% |