Background: 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.
Methods: Ten patients with IVLBCL diagnosed from June 2006 to January 2018 were retrospectively reviewed.
Results: 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.
Conclusion: 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.
Keywords: Hemophagocytosis; Intravascular large B-Cell lymphoma; Rituximab.
Copyright © 2020 Chang Gung University. Published by Elsevier B.V. All rights reserved.