Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma with a poor prognosis. This retrospective study reports the clinicopathological characteristics and outcomes of six cases of IVLBCL diagnosed in a period of 8 years in a tertiary hospital. The mean age was 66 years, with no sex predilection. Dizziness, gait instability, and hepatosplenomegaly were the most common presentations. Laboratory and brain imaging findings were inconclusive for lymphoma in all cases. Brain biopsies were diagnostic in two cases, while skin biopsies led to diagnosis in three patients. Only one patient with hemophagocytic variant was diagnosed at autopsy. Histologically, atypical lymphocytes infiltrated deep small- and medium-sized vessels, showing positive staining for B-cell markers. All patients who received combined chemotherapy achieved a complete response. After a median follow-up of 4 years, four of six patients remain alive. This lymphoma represents a diagnostic challenge, requiring a high index of suspicion and a multidisciplinary approach.
García et al. (2026) studied this question.