Secondary central nervous system lymphoma (<scp>SCNSL</scp>) in mantle cell lymphoma (<scp>MCL</scp>): Characteristics and risk factors in a Danish nationwide population‐based study

Secondary central nervous system (CNS) lymphoma (SCNSL) in mantle cell lymphoma (MCL) is associated with dismal outcomes, and risk factors are not well documented. This population-based study reports the incidence of SCNSL, risk factors and survival. All patients diagnosed with MCL in Denmark in 2010-2022 without confirmed CNS involvement at diagnosis were included. Data were retrieved from the Danish National Lymphoma Registry and medical records. In total, 873 patients (median age 70 years) were included. The 10-year cumulative incidence of SCNSL was 3.8% (95% confidence interval [CI] 2.4-5.2). Risk factors for SCNSL were blastoid/pleomorphic MCL (hazard ratio [HR] 3.55, 95% CI 2.05-6.14), Ki67 ≥30% (HR 5.47, 95% CI 1.86-16.10) and elevated lactatedehydrogenase (HR 4.35, 95% CI 1.91-9.91). The 5-year cumulative incidence of SCNSL for patients with blastoid/pleomorphic MCL was 9.9% (95% CI 4.0-15.7). Patients with high CNS international prognostic index (CNS-IPI) had a high 1-year incidence of SCNSL of 5.8% (95% CI 1.6-10.0). Median progression-free survival and overall survival were 1.8 months (95% CI 1.3-4.7) and 2.4 months (95% CI 1.3-8.9). In conclusion, SCNSL of MCL is rare and associated with poor survival. Blastoid/pleomorphic subtype and high CNS-IPI identified patients at high risk of SCNSL for whom upfront screening for CNS involvement should be considered.

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Journal
British Journal of Haematology
Published
2025-08-08
DOI
https://doi.org/10.1111/bjh.70082
Primary Topic
CNS Lymphoma Diagnosis and Treatment
Type
article
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article

Secondary central nervous system lymphoma (SCNSL) in mantle cell lymphoma (MCL): Characteristics and risk factors in a Danish nationwide population‐based study

Eliza A. Hawkes, Kirsten Grønbæk, Gabriella Cunsolo, Trine Trab et al.
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British Journal of Haematology
CNS Lymphoma Diagnosis and Treatment
article

Secondary central nervous system lymphoma (SCNSL) in mantle cell lymphoma (MCL): Characteristics and risk factors in a Danish nationwide population‐based study

Eliza A. Hawkes, Kirsten Grønbæk, Gabriella Cunsolo, Trine Trab, Elfriede Dall, Thomas Stauffer Larsen, Peter de Nully Brown, Tarec Christoffer El‐Galaly, Iman Chanchiri, Ahmed Ludvigsen Al‐Mashhadi, Christian Bjørn Poulsen, Laura Mors Haunstrup, Simon Husby, Sebastian Rasch, Mette Johansen, Torgerð Ranadóttir
article en

Abstract

Secondary central nervous system (CNS) lymphoma (SCNSL) in mantle cell lymphoma (MCL) is associated with dismal outcomes, and risk factors are not well documented. This population-based study reports the incidence of SCNSL, risk factors and survival. All patients diagnosed with MCL in Denmark in 2010-2022 without confirmed CNS involvement at diagnosis were included. Data were retrieved from the Danish National Lymphoma Registry and medical records. In total, 873 patients (median age 70 years) were included. The 10-year cumulative incidence of SCNSL was 3.8% (95% confidence interval [CI] 2.4-5.2). Risk factors for SCNSL were blastoid/pleomorphic MCL (hazard ratio [HR] 3.55, 95% CI 2.05-6.14), Ki67 ≥30% (HR 5.47, 95% CI 1.86-16.10) and elevated lactatedehydrogenase (HR 4.35, 95% CI 1.91-9.91). The 5-year cumulative incidence of SCNSL for patients with blastoid/pleomorphic MCL was 9.9% (95% CI 4.0-15.7). Patients with high CNS international prognostic index (CNS-IPI) had a high 1-year incidence of SCNSL of 5.8% (95% CI 1.6-10.0). Median progression-free survival and overall survival were 1.8 months (95% CI 1.3-4.7) and 2.4 months (95% CI 1.3-8.9). In conclusion, SCNSL of MCL is rare and associated with poor survival. Blastoid/pleomorphic subtype and high CNS-IPI identified patients at high risk of SCNSL for whom upfront screening for CNS involvement should be considered.

British Journal of HaematologyVol. 207(4)
University of Copenhagen (DK), La Trobe University (AU), Aarhus University (DK), Aalborg University Hospital (DK), Odense University Hospital (DK), Aarhus University Hospital (DK), Rigshospitalet (DK), Zealand University Hospital (DK), Vejle Sygehus (DK), Regionshospitalet Herning (DK), Olivia Newton-John Cancer Wellness & Research Centre (AU), Austin Health (AU), Monash University (AU)
Bristol-Myers Squibb, AstraZeneca, Gilead Sciences, Celgene, F. Hoffmann-La Roche, Kræftens Bekæmpelse, Regeneron Pharmaceuticals, Rigshospitalet, Swedish Orphan Biovitrum, William Demant Fonden, Science and Engineering Research Board, Cilag
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Openalex Percentile: Top 70%
CNS Lymphoma Diagnosis and Treatment
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