Trends in the Mortality of Enteropathy-Associated T-cell Lymphoma in the US, 2000-2020: A Population-Based Analysis

Introduction: Enteropathy-associated T-cell lymphoma (EATL) is a type of non-Hodgkin lymphoma that occurs in the intestinal tract. It is a rare but lethal tumor with a reported median survival time of approximately 10 months. Due to the limited data available, we conducted a time trend analysis of EATL mortality rates in the US. Methods: Using data from the Surveillance, Epidemiology, and End Results (SEER) database, we assessed the incidence-based mortality (IBM) and survival rates of EATL between 2000 and 2020. We calculated age-adjusted IBM rates per 1,000,000 population, and IBM rate ratios (RRs) for different age groups, genders, and races using SEER*Stat program version 8.4.1.2. We also retrieved overall and summary stage-specific survival rates. We analyzed overall, and age- and sex-specific trends of the IBM rates of EATL using Joinpoint Regression program version 5.0.2, and calculated annual percentage change (APC) and average annual percentage change (AAPC). A P-value < 0.05 was considered statistically significant. Results: From 2000 to 2020, a total of 326 EATL-specific deaths occurred with an annual IBM rate of 0.116 per 1,000,000. Overall, the IBM rate of EATL increased 59.49% per year between 2000 and 2003 (P < 0.05) but remained stable after that (APC 1.66%; P >0.05) with an AAPC of 8.77% (95% CI: 4.07%-15.84%; P < 0.05) (Figure 1). The IBM rates for patients with ages less than 60 years remained stable (AAPC 0.06%; P >0.05) while for those aged 60 years and older, the IBM rates increased by an average of 11.76% per year (AAPC difference 11.70%; P < 0.05). Females had a significantly lower IBM as compared to males (RR 0.52, 95% CI: 0.42-0.66; P < 0.001). When compared to Whites, only Asians or Pacific Islanders had significantly higher IBM rates (RR 1.81, 95% CI: 1.27-2.53; P < 0.05) while Blacks and American Indians/Alaska Natives had similar rates. The observed age-standardized overall 1- and 5-year survival rates were 37.9% and 17.1%, respectively. The 5-year survival rates for localized, regional, and distant cancers were 26.0%, 18.6%, and 10.2%, respectively. Conclusion: Although the overall IBM of EATL has remained stable after 2003, there is an upward trend in mortality rates in elderly patients (>60 years) with a large increase in IBM per year. Additionally, male sex and Asian or Pacific Islander race are associated with worse prognosis.Figure 1.: Age-adjusted incidence-based mortality rates per 1,000,000 population for enteropathy-associated T-cell lymphoma (EATL) in the US between 2000 and 2020.