A retrospective study of treatment and outcomes in synchronous systemic and central nervous system large B-cell lymphoma

Synchronous systemic and de novo secondary central nervous system (CNS) large B-cell lymphoma (LBCL) is an aggressive clinical entity with a historically poor prognosis. Given the rarity of this presentation, prospective studies are limited, and treatment paradigms and outcomes are extrapolated from small, heterogenous retrospective studies. We performed a retrospective study with extended follow-up for 63 consecutive patients with previously untreated synchronous systemic and de novo secondary CNS LBCLs presenting to 2 institutions over 21 years. Most patients had diffuse LBCL (73%) and were treated with an average of 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, Oncovin [vincristine], and prednisone) intercalated with high-dose IV methotrexate (R-CHOP-M). The overall response rate was 84% (75% complete). With a median follow-up of 8.1 years, the median progression-free survival (PFS) was 1.2 years, and the 6-year PFS rate was 37%. The median overall survival (OS) was 7.9 years, and the 6-year OS rate was 52%. Normal lactate dehydrogenase (LDH), low International Prognostic Index (IPI) score, and brain parenchymal-only CNS disease were associated with improved PFS, whereas normal LDH and parenchymal disease were associated with OS. The 25% of patients who underwent consolidation with high-dose chemotherapy (HDC) and autologous stem cell transplant (ASCT) had superior PFS and OS than patients who did not receive a transplant, with particular benefit in those with IPI score of ≥3. This study demonstrates that a proportion of patients presenting with secondary CNS involvement are cured with upfront chemoimmunotherapy with or without HDC/ASCT and helps identify prognostically favorable subgroups, which can guide counseling of patients with this rare, high-risk clinical presentation.

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Publication Details

Journal
Blood Advances
Published
2025-10-31
DOI
https://doi.org/10.1182/bloodadvances.2025017127
Primary Topic
CNS Lymphoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
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article

A retrospective study of treatment and outcomes in synchronous systemic and central nervous system large B-cell lymphoma

Jeremy S. Abramson, Robert Allyn Redd, Ronald W. Takvorian, Lakshmi Nayak et al.
Blood Advances
CNS Lymphoma Diagnosis and Treatment
article

A retrospective study of treatment and outcomes in synchronous systemic and central nervous system large B-cell lymphoma

Jeremy S. Abramson, Robert Allyn Redd, Ronald W. Takvorian, Lakshmi Nayak, Isabel Arrillaga‐Romany, Jeffrey A. Barnes, J Ricardo McFaline-Figueroa, Ephraim P. Hochberg, Philipp Karschnia, J. Erika Haydu, Caron Alyce Jacobson, Matthew M. Lei, April F. Eichler, Jörg Dietrich, Philippe A. Armand, Ugonma Nnenna Chukwueke, Justin T. Jordan, Scott Randall Plotkin, Patrick Connor Johnson, Eric D. Jacobsen, Ann Steward LaCasce, Jennifer Leigh Crombie, Jacob D. Soumerai
article en

Abstract

Synchronous systemic and de novo secondary central nervous system (CNS) large B-cell lymphoma (LBCL) is an aggressive clinical entity with a historically poor prognosis. Given the rarity of this presentation, prospective studies are limited, and treatment paradigms and outcomes are extrapolated from small, heterogenous retrospective studies. We performed a retrospective study with extended follow-up for 63 consecutive patients with previously untreated synchronous systemic and de novo secondary CNS LBCLs presenting to 2 institutions over 21 years. Most patients had diffuse LBCL (73%) and were treated with an average of 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, Oncovin [vincristine], and prednisone) intercalated with high-dose IV methotrexate (R-CHOP-M). The overall response rate was 84% (75% complete). With a median follow-up of 8.1 years, the median progression-free survival (PFS) was 1.2 years, and the 6-year PFS rate was 37%. The median overall survival (OS) was 7.9 years, and the 6-year OS rate was 52%. Normal lactate dehydrogenase (LDH), low International Prognostic Index (IPI) score, and brain parenchymal-only CNS disease were associated with improved PFS, whereas normal LDH and parenchymal disease were associated with OS. The 25% of patients who underwent consolidation with high-dose chemotherapy (HDC) and autologous stem cell transplant (ASCT) had superior PFS and OS than patients who did not receive a transplant, with particular benefit in those with IPI score of ≥3. This study demonstrates that a proportion of patients presenting with secondary CNS involvement are cured with upfront chemoimmunotherapy with or without HDC/ASCT and helps identify prognostically favorable subgroups, which can guide counseling of patients with this rare, high-risk clinical presentation.

Blood AdvancesVol. 10(2)
Harvard University (US), Friedrich-Alexander-Universität Erlangen-Nürnberg (DE), Massachusetts General Hospital (US), Universitätsklinikum Erlangen (DE), Dana-Farber Cancer Institute (US), Center for Cancer Research (US), Dana-Farber Brigham Cancer Center (US), New York University (US)
Amgen, Eli Lilly and Company, AstraZeneca, GlaxoSmithKline, Sanofi, Massachusetts General Hospital, American Society of Clinical Oncology, Les Laboratories Pierre Fabre, Adaptive Biotechnologies, BeiGene, Moderna, Genentech
Openalex Percentile: Top 55%
CNS Lymphoma Diagnosis and Treatment
DOIOpen Access PDFOpenAlex
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