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American Journal of Hematology


Background: EATL is very rare and accounts for less than 1 percent of all non-Hodgkin lymphomas. EATL occurs most commonly in patients with celiac disease and carries a uniformly poor prognosis. The optimal treatment for EATL is unclear and the data is limited to case reports and small retrospective studies.

Methods: ICD-O-3 (9717) histological code was used to identify cases of EATL from the population-based cancer registries of the Surveillance Epidemiology and End Results program (SEER) between 2006 and 2016. Frequency, demographics, and survival data were assessed using SPSS statistical software.

Results: A total of 138 cases were found. The median age of diagnosis was 66. EATL was more common in men (54.3%) and in Caucasians (76.8%). Of all EATL cases, 53.6% received chemotherapy and only 3.6% received radiation therapy. EATL was the cause of death in 58 % of the cases. Median overall survival (OS) was 3 months; 95% CI, (1.4 to 4.5 months) and disease specific overall survival (DSOS) was 8 months; 95% CI, (3.8 to 12.1 months). Chemotherapy significantly improved both OS (7 months vs 1 month; P< 0.001) and DSOS (12 months vs 6 months; P=0.005). Multivariable analysis demonstrated that age, sex, race and radiation therapy were not associated with mortality. Chemotherapy was associated with decreased mortality risk (HR, 0.48; CI, 0.32 -0.71; p< 0.001)

Conclusions: EATL is a rare subtype of non-Hodgkin lymphoma. Despite the advances in treating non-Hodgkin lymphomas, EATL prognosis remains poor. In concordance with previously reported data, chemotherapy has shown to mitigate the poor prognosis associated with EATL in our study.





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