Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study.

in Annals of neurology by Elodie Berthet, Antoine Guillonnet, Caroline Houillier, Renata Ursu, Carole Soussain, Mehdi Touat, Antoine Gueguen, Benoît de Renzis, Kevin Bigaut, Guido Ahle, Pierre Durozard, Deborah Grosset-Janin, Lucie Oberic, Antoine Bonnet, Anne-Pascale Grandjean, Cécile Moluçon-Chabrot, Khê Hoang-Xuan, Hugues Chabriat, Stéphanie Guey

TLDR

  • The study identified key phenotypic features for early diagnosis of intravascular lymphoma with CNS involvement, including focal neurological episodes, brain MRI abnormalities, and biomarkers, which could improve patient outcomes.

Abstract

Intravascular 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. We 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. In 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. The results of this study highlight "red flags" that could help accelerate the diagnosis of intravascular lymphoma involving the CNS. ANN NEUROL 2024.

Overview

  • The study aimed to identify key phenotypic features for early diagnosis of intravascular lymphoma with CNS involvement through a multicenter retrospective cohort study.
  • The study analyzed data from 17 patients recruited in collaboration with the French Expert Network for Oculo-Cerebral Lymphomas (LOC network) and retrospectively reviewed medical records.
  • The primary objective of the study was to identify early diagnostic signs and symptoms of intravascular lymphoma with CNS involvement to improve patient outcomes.

Comparative Analysis & Findings

  • The study found that 88% of patients developed focal neurological episodes with a sudden onset, and 15 of 17 patients experienced rapid cognitive deterioration.
  • Brain MRI showed polymorphic FLAIR hyperintensities in 87% of patients, and DWI-positive lesions in 81% of patients, which had unusual characteristics for ischemic lesions.
  • Early-onset inflammatory syndrome, elevated lactate dehydrogenase (LDH) levels, and an increased interleukin 10/6 ratio over 0.7 were observed in over 90% of cases.

Implications and Future Directions

  • The study's findings highlight 'red flags' that could help accelerate the diagnosis of intravascular lymphoma involving the CNS, which is often delayed due to the lack of distinctive features.
  • Future studies should focus on developing effective diagnostic criteria and treatment protocols for intravascular lymphoma with CNS involvement.
  • The study suggests that skin biopsy could be a useful diagnostic tool, and further investigation is needed to explore its potential utility.
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