Prognosis of Intravascular Large B Cell Lymphoma (IVLBCL): Analysis of 182 Patients from Global Case Series

PURPOSE: This study provides an overview of the prognosis of intravascular large B cell lymphoma (IVLBCL) over the past 10 years and analyzes the possible relevant factors. PATIENTS AND METHODS: We conducted a literature search of case reports, case series, and retrospective studies of IVLBCL published from January 2008 to July 2018. After excluding inappropriate data, 103 publications were selected for the analysis. Statistical analyses of different treatment modalities, the effect of blood–brain barrier (BBB)-penetrating drugs, and prognostic factors for outcomes were performed. RESULTS: In total, 182 pathologically confirmed cases of IVLBCL were included in our study. The results revealed that the 1- and 3-year overall survival rates were 42.3 and 11.5%, respectively, whereas the median overall survival was 340 days. Overall survival (450 days vs 180 days) and progression-free survival (420 days vs 150 days) were significantly longer in patients who received rituximab-containing regimens than in those treated with other regimens. For IVLBCL involving the CNS, regimens containing BBB-penetrating drugs failed to provide an additional survival benefit. In addition, lactic dehydrogenase levels ≥700 U/L, CNS involvement, and hemophagocytic syndrome were identified as unfavorable risk factors in patients with IVLBCL, whereas skin involvement appeared to be a protective factor. CONCLUSION: Rituximab-containing chemotherapy can improve the outcomes of patients with IVLBCL, but the prognosis remains unsatisfactory. Treatment regimens containing BBB-penetrating drugs failed to improve outcomes in patients with CNS-involved IVLBCL. Several factors affect the prognosis of patients with IVLBCL, and further research on the underlying mechanisms is needed.