A population-based study of intravascular large B-cell lymphoma (IVLBCL) in the US was conducted to determine incidence, demographic and clinical characteristics, prognostic factors, and survival compared with diffuse large B-cell lymphoma not otherwise specified (DLBCL NOS). In the years 2000-2013, the age-adjusted incidence rate of IVLBCL was 0.095 (case/1,000,000). The incidence of IVLBCL increased significantly from 2000 to 2013 (annual percentage change = 9.84, p = 0.002). We identified 344 patients with IVLBCL. The median age at diagnosis was 70 years. The median 1, 3, and 5-year OS rates for IVLBCL were 66.2, 51.8, and 46.3%, respectively. After propensity matching, the 5-year overall survival of IVLBCL was comparable to DLBCL NOS (46.4 versus 46.5%, p = 0.53). On multivariate analysis advanced age, advanced stage and having Medicaid or Medicare insurance predicted worse OS, whereas female gender and use of radiation therapy predicted better OS.
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