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Intravascular large B‐cell lymphoma is a rare disease of the large B cells characterized by selective growth in the lumina of small vessels in systemic organs. Since first reported in 1959, the difficulty of obtaining sufficient tumor cells from biopsy specimens has hampered the elucidation of its underlying biology. Recent progress using xenograft models and plasma cell‐free DNA has uncovered genetic features that are similar to those of activated B‐cell type diffuse large B‐cell lymphoma, including  MYD88  and  CD79B  mutations and frequent alterations in immune check point‐related genes such as  PD‐L1  and  PD‐L2 . Given the improvement in clinical outcomes and a higher risk of secondary central nervous system (CNS) involvement in the rituximab era, a phase 2 trial of R‐CHOP combined with high‐dose methotrexate and intrathecal chemotherapy as a CNS‐oriented therapy has been conducted. This trial, the PRIMEUR‐IVL study, has displayed good progression‐free survival and a low cumulative incidence of secondary CNS involvement. Long‐term follow‐up within this trial is still ongoing. Further understanding of the pathophysiology of the disease and improvements in clinical outcomes are still needed.

Current progress and future perspectives of research on intravascular large B‐cell lymphoma