High-grade B-cell lymphoma, not otherwise specified: CNS involvement and outcomes in a multi-institutional series

•CNS involvement is uncommon in HGBL NOS but highly prognostic for future CNS recurrence.•CNS recurrence was higher in patients with HGBL with blood or marrow involvement, CD5 expression, non– germinal center B-cell cell of origin, and “dual-expresser lymphoma” phenotype. [Display omitted] Little is...

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Published in:Blood advances 2024-08
Main Authors: Epperla, Narendranath, Zayac, Adam S., Landsburg, Daniel J., Bock, Allison M., Nowakowski, Grzegorz S., Ayers, Emily C., Girton, Mark, Hu, Marie, Beckman, Amy, Li, Shaoying, Medeiros, L. Jeffrey, Chang, Julie E., Kurt, Habibe, Sandoval-Sus, Jose, Ansari-Lari, Mohammad Ali, Kothari, Shalin K., Kress, Anna, Xu, Mina L., Torka, Pallawi, Sundaram, Suchitra, Smith, Stephen D., Naresh, Kikkeri N., Karimi, Yasmin, Bond, David A., Evens, Andrew M., Naik, Seema G., Kamdar, Manali, Haverkos, Bradley M., Karmali, Reem, Farooq, Umar, Vose, Julie M., Rubinstein, Paul, Chaudhry, Amina, Olszewski, Adam J.
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Language:English
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Summary:•CNS involvement is uncommon in HGBL NOS but highly prognostic for future CNS recurrence.•CNS recurrence was higher in patients with HGBL with blood or marrow involvement, CD5 expression, non– germinal center B-cell cell of origin, and “dual-expresser lymphoma” phenotype. [Display omitted] Little is known about the central nervous system (CNS) risk in high-grade B-cell lymphoma, not otherwise specified (HGBL NOS). Hence, we sought to describe the rates of baseline CNS involvement, risk of CNS recurrence after primary therapy, and management strategies in HGBL NOS. In this multicenter retrospective study, we included 160 adults with newly diagnosed HGBL NOS treated between 2016 and 2021 at 20 US institutions. Eleven patients (7%) had baseline CNS involvement at diagnosis (leptomeningeal = 6, parenchymal = 4, and both = 1). Baseline CNS involvement was significantly associated only with MYC rearrangement (odds ratio [OR] = 3.5) and testicular (in men) or female pelvic (in women) involvement (OR = 8.1). There was no significant difference in survival outcomes between patients with HGBL NOS with (median progression-free survival [PFS] = 4 years) or without (median PFS = 2.4 years) baseline CNS involvement (P = .45). The cumulative incidence of CNS recurrence at 3 years was 11%. Patients with baseline CNS involvement were at the highest risk (48.5% vs 8% for those without baseline CNS involvement) and were excluded from the risk factors analysis for CNS recurrence. The risk for CNS recurrence was significantly associated with blood or bone marrow involvement, CD5 expression, non–germinal center B-cell subtype, and “dual-expresser lymphoma” phenotype, however, high CNS International Prognostic Index was not. The prognosis of relapsed HGBL NOS was poor, regardless of whether recurrence was systemic or limited to the CNS, and with currently available salvage strategies, including autologous transplantation and chimeric antigen receptor T-cell modalities, almost all patients with CNS recurrence ultimately succumbed to their disease. These patients represent an unmet need and should be prioritized for experimental approaches.
ISSN:2473-9529
2473-9537
2473-9537
DOI:10.1182/bloodadvances.2024013791