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.
Background: Primary cutaneous diffuse large B cell lymphoma-leg type (DLBCL-LT) is relatively uncommon lymphoma accounting for about 1 to 3 % of all cutaneous lymphoma and about 10-20% of primary cutaneous B cell lymphomas. There is data for survival outcomes in primary cutaneous B cell lymphoma in general population after introduction of rituximab but little is known about outcomes in DLBCL-LT patients in general population. The available data on survival in DLBCL-LT are from small case series and single institution studies. We conducted this study to evaluate the survival outcome in patients with PCDLBCL-LT after introduction of rituximab. Methods: We utilized SEER 18 database to select adult (18+ years) patients diagnosed from 2004 to 2014 with ICD-3-0 code of 9680/3 (diffuse large B cell lymphoma, not otherwise specified)with localized stage by summary stage 2000 with skin as the primary site of disease to identify patient with DLBCL-LT. Patients were divided into various cohorts based on age (<65 versus 65+ years), sex, race (Caucasians, African American and Others) and median household income of county of residence (< $50,00 versus ≥ $50,000). We utilized SEER*stat to calculate age adjusted incidence rate using 2000 US standard population. Kaplan Meyer curve was utilized to calculate 5-year overall survival. Cox proportional hazard model was used for multivariate analysis of factor associated with survival. Result: A total of 485 patients were identified with DLBCL-LT as the only or first primary cancer case. The median age at diagnosis was 70 years. Its incidence was found to be about 0.09 case per 100,000 population with incidence in males being about 1.6 times that of females. Majority of patient included in our study were males (52 %), older (59.4%), and Caucasians (85.6%). 5-year overall survival was found to be 59%. Older patients were found to have significantly worse 5-year overall survival compared to younger patients with HR of 7.9 with 95% CI of 4.9 to 12.7. There was no disparity in cancer outcome based on race, sex or median income of the county of residence. When we compared the outcome of patients diagnosed in earlier 5 years to later 6 years of the study period, the survival curves almost overlapped suggesting that there has been little if any improvement in outcome in recent years. Conclusion: Our study showed that about 60% of patients with DLBCL-LT live for 5 years in real world setting which is comparable to the outcome reported by other studies on outcome in primary cutaneous B cell lymphoma. Even though this is disease of elderly, as the median age at diagnosis is 70 years, the outcome in older population was found to be significantly worse compared to younger patients. Given this is relatively uncommon disease, we recommend large registry-based studies to try to improve outcome in these patient population. Disclosures No relevant conflicts of interest to declare.
Background: Intravascular large B cell lymphoma (IVLBCL) is a rare and aggressive non-Hodgkin lymphoma lacking any prior US based population studies. We aimed to conduct a population based study of IVLBCL in the US and compare characteristics and survival to diffuse large B cell lymphoma, not otherwise specified (DLBCL, NOS). Methods: We used Surveillance, Epidemiology, and End Results (SEER) registries from 2000 to 2013 to calculate incidence rates (case/1,000,000) and age-adjusted to the US 2000 standard population. We collected patient level data from the NCDB Participant User Files using ICD-O-3 histology code 9680 with ICD-O-3 primary site modifier C499 and analyzed for demographic and clinical characteristics as well as overall survival (2004-2013). Chi-Square test was used to compare the characteristics of patients with IVLBCL to that of DLBCL, NOS. Overall survival analysis was performed using Kaplan- Meier method and compared by log rank. Overall survival (OS) of IVLBCL was compared to propensity-matched cohort of DLBCL, NOS. Multivariate analysis was conducted using weighted Cox Proportional Hazard regression model. Results: In the years 2000-2013, the overall crude incidence rate of IVLBCL was 0.093 (case/1,000,000) and age-adjusted incidence rate of IVLBCL was 0.095 (case/1,000,000). A total of 388 IVLBCL patients were identified in NCDB database. Patients with IVLBCL (n=388), compared to DLBCL, NOS (n=155,436), were more likely to be of age ≥ 65 (64.2% vs 56.9%, p=0.0039), female (51.8% vs 46.5, p=0.036), diagnosed at advanced stage (stage IV, 51.5% vs 33.4%, p<0.0001) and less likely to receive chemotherapy (70.4% vs 78.9%, p = 0.0001).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 (46.6% vs 45.4%, p=0.61). On multivariate analysis, advanced age (HR 1.03, 95% CI 1.032-1.034; p<0.0001), advanced stage (Stage III: HR 1.34, 95% CI 1.31-1.38; <0.0001, Stage IV: HR 1.81, 95% CI 1.77-1.85; p<0.0001), having Medicaid (HR 1.72, 95% CI 1.66-1.79; p<0.001) or Medicare (HR 1.24, 95% CI 1.20-1.26; p<0.0001) predicted worse OS, whereas female gender (HR 0.86, 95% CI 0.85-0.88; p<0.0001) and use of radiation therapy (HR 0.76, 95% CI 0.75-0.78; p<0.0001) predicted better OS. Conclusions: This is the largest series of IVLBCL reported to date. Our study provides characteristics and OS of a large cohort of this rare entity, which can be used for patient education and may provide background information for future research. In the Rituximab era, the OS of patients with IVLBCL is comparable to DLBCL, NOS. In addition, our study also reports predictors of OS in patients with IVLBCL. Disclosures No relevant conflicts of interest to declare.
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