ArticleAnnals of neurology2025
Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study.
Article in Annals of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- A 13-Year Review of Non-Diffuse Large B Cell Lymphomas of the Central Nervous System at a Tertiary Hospital: A Case Series Study.Neuropathology : official journal of the Japanese Society of Neuropathology · 2026Article
- Intravascular Lymphoma Associated with the Female Genital Tract-Diagnostic Considerations, Therapeutic Approaches, and Outcomes.Diseases (Basel, Switzerland) · 2026Review
- Case Report: Probable CLIPPERS in a patient with cutaneous T-cell lymphoma in remission: 5-year follow-up.Frontiers in immunology · 2026Article
- Intravascular large B-cell lymphoma presenting as encephalomyelitis diagnosed by renal biopsy: a case report.BMC neurology · 2025Article
- Review
- Intravascular Large B-Cell Lymphoma Diagnosed After Recurrent Stroke: Case Report and Literature Review.Neurology international · 2025Article
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Authors and funding
19 authors.
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Abstract
objectiveIntravascular lymphoma is a rare subtype of B-cell lymphoma characterized by a clonal proliferation restricted to the lumen of small vessels. Over 50% of patients exhibit central nervous system (CNS) involvement, but diagnosis is often delayed due to the lack of distinctive features. We aimed to identify key phenotypic features for early diagnosis of intravascular lymphoma with CNS involvement through an in-depth cohort study.
methodsWe built up a multicenter retrospective cohort of 17 patients recruited in collaboration with the French Expert Network for Oculo-Cerebral Lymphomas (LOC network), and retrospectively analyzed data from medical records.
resultsIn this cohort, 15 of 17 (88%) patients developed focal neurological episodes, often fluctuating and/or recurrent, with a sudden onset in 68% of episodes, suggesting a vascular origin. Rapid cognitive deterioration occurred in 15 of 17 (88%) patients, psychiatric manifestations in 8 of 17 (47%), and "B signs" in 14 of 17 (82%). Brain MRI showed polymorphic FLAIR hyperintensities in 14 of 16 (87%) patients, and DWI-positive lesions in 13 of 16 (81%) of patients, which accumulated over time and had unusual characteristics for ischemic lesions (progressive growth, persistent DWI-hyperintensity over 1 month, surrounded by a wider FLAIR hyperintensity). Early-onset inflammatory syndrome, and elevated lactate dehydrogenase (LDH) levels were observed in over 90% of cases. Mild and inconsistent meningitis contrasted with a nearly-constant hyperproteinorachia. An increased interleukin 10/6 ratio over 0,7 was found in 4 of 7 (57%) patients, and skin biopsy led to a pathological diagnosis in 3 of 6 (50%) patients.
interpretationThe results of this study highlight "red flags" that could help accelerate the diagnosis of intravascular lymphoma involving the CNS. ANN NEUROL 2025;97:435-448.
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