Document Type

Article

Publication Date

10-15-2020

Publication Title

American Journal of Hematology

Abstract

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.

Volume

95

Issue

S1

First Page

S3

Last Page

S3

DOI

10.1002/ajh.26005

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