Recent advances in understanding and management of intravascular large B-cell lymphoma.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by the proliferation of tumor cells within the lumina of small vessels in systemic organs. Diagnosis remains challenging due to the frequent presentation of non-specific symptoms such as fever and general malaise. Since its listing as a distinct disease entity in the 4th edition of the World Health Organization classification, awareness of IVLBCL has increased, leading to improvements in diagnostic accuracy. Nevertheless, patients with delayed or difficult diagnosis continue to be reported. Recent genetic studies have identified recurrent genetic abnormalities associated with IVLBCL, and the potential utility of liquid biopsy for diagnosis and treatment monitoring has been suggested. However, despite these advances, a gap remains between translational research and clinical application, particularly regarding how liquid biopsy can be incorporated into clinical practice. Regarding treatment, although standard chemotherapy with central-nervous-system-directed therapy has demonstrated favorable outcomes in initial management, the efficacy and safety of emerging immune cell therapies in IVLBCL remain largely unknown. This article aims to review recent advances in the understanding and management of IVLBCL, with the ultimate goal of improving clinical outcomes and advancing therapeutic management of IVLBCL.