Clinical features and prognostic factors of intravascular large B-cell lymphoma: a cohort study of 20 patients from 2018 to 2024

Abstract Background Intravascular large B-cell lymphoma (IVLBCL) is a rare, aggressive lymphoma characterized by lymphoma cells proliferating within small blood vessels, lacking an extravascular mass. Its low incidence complicates diagnosis and treatment. Methods This study analyzed 20 IVLBCL patients diagnosed between 2018 and 2024, aiming to describe their clinical presentations, diagnostic procedures, treatment, and outcomes. Results All patients had stage IV disease with poor ECOG performance status and high-risk IPI scores (100% ≥ 4). Common symptoms included fever (65%), impaired consciousness (30%), and respiratory issues (25%). Laboratory findings revealed cytopenias (60% anemia, 60% thrombocytopenia, and 35% leukocytopenia), elevated lactate dehydrogenase (20/20, 100%), C-reactive protein (14/14, 100%), and erythrocyte sedimentation rate (11/12, 91.7%). Five of six patients had extreme IL-10 elevation. Imaging studies showed a high incidence of adrenal gland involvement (9/20, 45%), reproductive system involvement (7/20, 35%), central nervous system (CNS) involvement (6/20, 30%), and splenomegaly (9/20, 45%). Nineteen of 20 patients were non-GCB (CD10-MUM1+). Seven of 14 patients tested positive for CD5. Eighteen of 20 patients received CD20 antibody plus chemotherapy as first-line therapy, with complete responses (CRs) achieved in 7 of 18 patients. After a median follow-up of 42.4 months, the 1-year and 2-year overall survival rates were 71.3% and 65.4%, respectively. Unfavorable risk factors included central nervous system (CNS) and bone marrow (BM) involvement, and elevated creatinine. Conclusion The study highlights the high incidence of adrenal and reproductive system involvement in IVLBCL, with CNS, BM, and elevated creatinine contributing to rapid disease progression.