To study clinical, laboratory, imaging findings and prognosis of patients with intravascular lymphoma (IVL) associated with stroke.

IVL is a rare, aggressive systemic large B-cell lymphoma in which lymphoma cells selectively involve lumina of blood vessels, particularly small and medium-sized, and rarely involves brain parenchyma. Stroke is a common manifestation of this rare disease apart from cognitive impairment.

A systematic review was done using Pubmed and Medline database from incident to August 2023 using the search terms intravascular large B-cell lymphoma, intravascular lymphoma, and stroke.

There were 58 cases with confirmed diagnosis of IVL associated with stroke with mean age 62.9 ± 9.6 years (female 50%). Classical lateralizing stroke symptoms was noted in only 69.2% cases (n 52). B-symptoms was present in 19.6% cases (n 51). Common serum abnormalities included elevated lactate dehydrogenase (LDH) (96.6%; n 29), interleukins (85.7%; n 7), and erythrocyte sedimentation rate (ESR) (79%; n 19). Common cerebrospinal fluid analysis finding was elevated protein (45.7%; n 35) and positive lymphoma associated flow cytometry was noted in only 36.3% cases (n 11).

53 cases brain magnetic resonance imaging (MRI) showed either diffusion restriction (39.6%) or abnormal T2-lesions (56.6%) which were mostly located in the subcortical regions (58.5%). Dynamic pattern of MRI lesions was noted in studies. In electroencephalogram (EEG) finding, background slowing was commonly noted (64%; n 12). 71.1% cases died (n 45). 30.8% were treated with R-CHOP chemotherapy, among whom 25% cases died.

Our study suggests, IVL associated stroke carry a high mortality rate and do not always present with classic stroke symptoms or restricted diffusion in brain MRI. Indicators such as associated B-symptoms, dynamic MRI DWI/T2-lesions primarily located in subcortical regions, elevated serum LDH, ESR, and interleukins can aid in early diagnosis apart from tissue biopsy and treatment.