Clinical characteristics, treatment, and long-term outcomes in 987 patients with splenic marginal zone lymphoma to inform treatment and surveillance strategies

Splenic Marginal Zone Lymphoma (SMZL) is an indolent non-Hodgkin lymphoma subtype. Due to its rarity, evaluation of real-world data is necessary to inform clinical practice. In this study, adults diagnosed with SMZL between 2000 and 2018 from North America, Europe, and Australia were included and followed until death or last visit. Data were extracted from medical records and regional/national registries. In total, 987 patients with median age of 68 years were included. The 10-year overall survival (OS) estimates were 59% (95%CI: 55-63) from first-line treatment and 49% (95%CI: 43-57) from second-line treatment. First-line splenectomy ( n = 434) and rituximab monotherapy ( n = 121) were associated with an Inverse Probability of Treatment Weighting (IPTW)-adjusted 5-year failure-free survival (FFS) of 52% for splenectomy versus 42% for rituximab; HR 0.70 (95%CI: 0.44-1.13) and adjusted HR for OS of 1.09 (95%CI: 0.59-2.01). The 10-year cumulative risk of transformation from diagnosis was 15% (95%CI: 12-18) with 5-year post-transformation OS of 38% (95%CI: 29-49), and 23% (95%CI: 13-41) for transformation after previous chemotherapy. Lower rates of transformation were seen in patients receiving frontline rituximab. While frontline therapy with either splenectomy or rituximab monotherapy had similarly favorable outcomes, patients had a persistent risk of progression and transformation andsurvival after transformation was poor.

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Journal
Blood Cancer Journal
Published
2026-11-09
DOI
https://doi.org/10.1038/s41408-026-01660-2
Primary Topic
Lymphoma Diagnosis and Treatment
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article
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article

Clinical characteristics, treatment, and long-term outcomes in 987 patients with splenic marginal zone lymphoma to inform treatment and surveillance strategies

Knut B. Smeland, Eliza Anne Hawkes, Andreea Sima, Simon Østergaard et al.
Blood Cancer Journal
Lymphoma Diagnosis and Treatment
article

Clinical characteristics, treatment, and long-term outcomes in 987 patients with splenic marginal zone lymphoma to inform treatment and surveillance strategies

Knut B. Smeland, Eliza Anne Hawkes, Andreea Sima, Simon Østergaard, Anne Ortved Gang, Ruxandra Maria Irimia, Karin Ekstrom Ekstroem Smedby, Thomas Matthew Habermann, Claire Maquet, Bente Arboe, Rose‐Marie Amini, Tarec Christoffer El‐Galaly, Lay She Ng, Jonathan R Day, Iman Chanchiri, Jennifer Bøgh Jørgensen, Diego R. Villa, Mikkel Runason Simonsen, Ahmed Ludvigsen Al‐Mashhadi, Toby Andrew Eyre, Christian Bjørn Poulsen, Martin Bjerregård Pedersen, Mathew S. Maurer, Katharine He Xing, Chan Yoon Cheah, Arushi Khurana, James R. Cerhan, Hervé Ghesquières, Alexandros Rampotas, Gita Thanarajasingam, Judith Trotman, Elizabeth Smyth, Stephen Opat, Steven Hwang
article en

Abstract

Splenic Marginal Zone Lymphoma (SMZL) is an indolent non-Hodgkin lymphoma subtype. Due to its rarity, evaluation of real-world data is necessary to inform clinical practice. In this study, adults diagnosed with SMZL between 2000 and 2018 from North America, Europe, and Australia were included and followed until death or last visit. Data were extracted from medical records and regional/national registries. In total, 987 patients with median age of 68 years were included. The 10-year overall survival (OS) estimates were 59% (95%CI: 55-63) from first-line treatment and 49% (95%CI: 43-57) from second-line treatment. First-line splenectomy ( n = 434) and rituximab monotherapy ( n = 121) were associated with an Inverse Probability of Treatment Weighting (IPTW)-adjusted 5-year failure-free survival (FFS) of 52% for splenectomy versus 42% for rituximab; HR 0.70 (95%CI: 0.44-1.13) and adjusted HR for OS of 1.09 (95%CI: 0.59-2.01). The 10-year cumulative risk of transformation from diagnosis was 15% (95%CI: 12-18) with 5-year post-transformation OS of 38% (95%CI: 29-49), and 23% (95%CI: 13-41) for transformation after previous chemotherapy. Lower rates of transformation were seen in patients receiving frontline rituximab. While frontline therapy with either splenectomy or rituximab monotherapy had similarly favorable outcomes, patients had a persistent risk of progression and transformation andsurvival after transformation was poor.

Blood Cancer Journal
Université Claude Bernard Lyon 1 (FR), Uppsala University (SE), University of Copenhagen (DK), University of Iowa (US), Oslo University Hospital (NO), The University of Sydney (AU), Mayo Clinic (US), Karolinska University Hospital (SE), Uppsala University Hospital (SE), University of British Columbia (CA), Carol Davila University of Medicine and Pharmacy (RO), The University of Western Australia (AU), Aarhus University (DK), Roskilde Sygehus (DK), Aalborg University Hospital (DK), Concord Repatriation General Hospital (AU), Odense University Hospital (DK), Sir Charles Gairdner Hospital (AU), Aarhus University Hospital (DK), Rigshospitalet (DK), Karolinska Institutet (SE), Zealand University Hospital (DK), Hôpital Lyon Sud (FR), Regionshospitalet Herning (DK), Oxford University Hospitals NHS Trust (GB), BC Cancer – Surrey (CA), Monash University (AU), Aalborg University (DK)
Lymphoma Diagnosis and Treatment
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