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Intravascular large B-cell lymphoma mimicking seronegative neuromyelitis optica spectrum disorder.

Created on 10 Jul 2026

Authors

Marc Waugh, Ravi Ambati, Thomas Quartermaine, Sarah Morgan, Benjamin Wood, Benjamin McGettigan, Nerissa Jordan

Published in

Practical neurology. Jul 09, 2026. Epub Jul 09, 2026.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare aggressive extra-nodal lymphoma characterised by malignant B-cells proliferating within small to medium blood vessels. Often termed the 'great mimicker', it can affect almost any organ with heterogeneous clinical features. Up to half of cases have central nervous system involvement, resembling inflammatory, autoimmune or vascular disease. We report a man in his 60s presenting with saddle paraesthesia, faecal incontinence and bilateral foot drop. MR scan of the spine showed longitudinally extensive transverse myelitis, multifocal supratentorial white matter lesions and optic neuritis. He was initially diagnosed with seronegative neuromyelitis optica spectrum disorder (NMOSD), which appeared to respond well to corticosteroids, plasma exchange and rituximab. However, 8 months, later he relapsed with progressive cytopenias, a rising serum lactate dehydrogenase and new cerebral infarcts despite intensified immunosuppression. Random skin biopsies diagnosed IVLBCL. This case shows that IVLBCL can mimic seronegative NMOSD and immunosuppression may mask the true diagnosis.

PMID:
42425889
Bibliographic data and abstract were imported from PubMed on 10 Jul 2026.

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