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

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.