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. 2025 Feb 25;9(4):893-905.
doi: 10.1182/bloodadvances.2024014404.

Incidence and characterization of secondary CNS lymphoma in 1972 patients with DLBCL: a Danish nationwide cohort study

Elisabeth Reuben Tolley  1   2 , Torsten Holm Nielsen  1   3   4 , Ditte Stampe Hersby  1 , Simon Østergaard  3   5 , Malin Rasmussen  6 , Michael Roost Clausen  7 , Ahmed Ludvigsen Al-Mashhadi  8 , Karina Moeslund Egeberg  9 , Laura Mors Haunstrup  10 , Christian Brieghel  1 , Carsten Utoft Niemann  1   2 , Tarec Christoffer El-Galaly  6   10 , Lars Møller Pedersen  2   3
Affiliations

Incidence and characterization of secondary CNS lymphoma in 1972 patients with DLBCL: a Danish nationwide cohort study

Elisabeth Reuben Tolley et al. Blood Adv. .

Abstract

Secondary central nervous system lymphoma (SCNSL) is a rare manifestation of diffuse large B-cell lymphoma (DLBCL) with a poor prognosis. We present updated data from a nationwide study on the incidence and clinical characteristics of SCNSL. The incidence of SCNSL was calculated considering death or relapse without SCNSL as competing risks. Risk factors associated with SCNSL were identified using a cause-specific Cox proportional hazards model. A total of 1972 patients with DLBCL were included, of which 68 (3.4%) experienced SCNSL at the first relapse. The crude 1- and 2-year cumulative incidence of SCNSL was 2.0% (95% confidence interval [CI], 1.5-2.7) and 2.6% (95% CI, 2.0-3.4), respectively. For patients with a high-risk central nervous system international prognostic index (CNS-IPI) score, the 1- and 2-year cumulative incidence was 6.4% and 7.5%, respectively. The number and location of extranodal (EN) sites were the most significant predictors of SCNSL. Specific EN sites associated with an increased risk were the bone marrow, heart, kidneys/adrenal glands, ovaries, testes, and uterus. The median overall survival (OS) after SCNSL was 3.2 months. SCNSL within 6 months after the end of treatment (EOT) was associated with a higher baseline CNS-IPI score and worse OS than SCNSL >6 months after EOT. Patients with a combination of low-risk CNS-IPI and late-onset SCNSL had the most favorable prognosis. In conclusion, updated real-world population-based data on SCNSL at first relapse, adjusted for competing risks, demonstrated a lower incidence of SCNSL than previously reported, with the number and location of EN sites being the most significant predictors of SCNSL.

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Conflict of interest statement

Conflict-of-interest disclosure: M.R.C. reports consultancy for AbbVie, AstraZeneca, Genmab, Gilead, Incyte, Janssen, and Roche, and has received funding for travel expenses from AbbVie, AstraZeneca, Genmab, Janssen, Pfizer, and Roche. A.L.A.-M. has received funding from Genentech Inc. C.B. has received honoraria from Octapharma and AstraZeneca. C.U.N. has received research grants and/or consultancy fees from AbbVie, AstraZeneca, Janssen, Lilly, Genmab, BeiGene, Octapharma, CSL Behring, MSD, and Takeda. L.M.P. received a research grant from AbbVie. The remaining authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
Consort diagram of the study cohort. EHR, electronic health record; MR-C, magnetic resonance imaging of the cerebrum; Pts, patients.
Figure 2.
Figure 2.
Incidence of SCNSL with other relapses and death as competing events.
Figure 3.
Figure 3.
Incidence of SCNSL according to CNS-IPI risk groups with other relapses and death as competing events.
Figure 4.
Figure 4.
OS after SCNSL diagnosis.
See this image and copyright information in PMC

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