[Treatment strategy for central nervous system involvement in intravascular large B-cell lymphoma].

Intravascular large B-cell lymphoma (IVLBCL) is a distinct disease entity of non-Hodgkin lymphoma which is characterized in the current WHO classification by the selective growth of tumour cells in the lumina of small vessels of various organs. This rare disease entity has a high incidence of central nervous system (CNS) involvement at diagnosis. Although several retrospective analyses indicate that the treatment of this lymphoma has improved in the post-rituximab era, risk of CNS recurrence at 3 years still reaches ~25%. Considering the poor prognosis of IVLBCL patients with CNS recurrence, elucidating the optimal treatment strategies for this serious complication is vital in order to further improve its clinical management. Meanwhile, the efficacy of CNS prophylaxis for specific subtypes of lymphoma, for example Burkitt lymphoma and acute lymphocytic leukemia/lymphoblastic lymphoma, has been established, and several risk factors of CNS relapse for diffuse large B-cell lymphoma (DLBCL) were indicated by previous analyses. Considering that almost all IVLBCL patients have similar risk factors, CNS prophylaxis could also be effective, and thereby give rise to better clinical outcomes, in the treatment of IVLBCL, especially for patients without CNS involvement at initial diagnosis. Intensity regimens for CNS and systemic chemotherapy could be useful for patients with CNS involvement at initial diagnosis. This review describes the current understanding of clinical outcome and CNS involvement in IVLBCL, and discusses the optimal treatment strategy and future perspectives for CNS involvement of this rare lymphoma.