Intravascular Large B-cell lymphoma: A case series and review of literatures

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma with uncommon clinical presentations and poor prognosis. The purpose of this study is to report the clinical features and outcome of IVLBCL in a single institution of Taiwan. Ten patients with IVLBCL diagnosed from June 2006 to January 2018 were retrospectively reviewed. The median age was 61 (range 39–88) years. The most common presentation was fever (90%), cytopenia (90%), and confusion (50%). For all patients, the median progression free survival (PFS) and overall survival (OS) were 12.6 (95% confidence interval [CI] 0.0–76.1) and 18.8 (95% CI 0–59.3) months, respectively. Six patients received rituximab combined chemotherapy, and the other one patient was treated with chemotherapy alone. Six of seven (85.7%) patients achieved complete response after chemotherapy. The median PFS and OS for six patients who completed treatment were not reached. Three-year PFS and OS rates were 80% and 75%, respectively. Our study showed that patients might achieve durable remission after rituximab-based chemotherapy. The outcome of IVLBCL patients may further improve if early diagnosis and prompt treatment were made.