title: Treatment response in enteropathy associated T-cell lymphoma; survival in a large multicenter cohort.
abstract: Enteropathy-associated T-cell lymphoma (EATL) is a T-cell Non-Hodgkin Lymphoma which is highly associated with celiac disease. The prognosis of EATL has been considered poor and there are no standardized treatment protocols. Here, we evaluate treatment response and survival of EATL patients in a large multicenter cohort. A total of 61 patients diagnosed with EATL were analyzed. Various treatment regimens were applied in EATL during the past fifteen years including either monotherapy consisting of chemotherapy or resection, or combination therapy with both aforementioned regimens whether or not combined with stem-cell transplantation (SCT). Overall, 50/61 patients (82%) died after a median of 7.4 months. One- and five-year overall survival was 40 and 11%, respectively. Median follow-up in the survivors was 26 months. Patients treated with the most aggressive treatment, that is, resection, chemotherapy and autologous SCT, showed the most favourable outcome with complete remission in all patients, the lowest relapse rate and one- and five-year overall survival of 100 and 33%, respectively, although overall survival in this group was not significantly better as compared to patients treated with surgery and chemotherapy. This study indicates that combination treatment is superior compared to monotherapy. Whether or not consolidation therapy with autologous SCT may improve survival needs to be substantiated in a larger randomized international trial.
authors.affiliation: Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Petula
authors.initials: P
authors.lastname: Nijeboer
authors.affiliation: Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Laura R
authors.initials: LR
authors.lastname: de Baaij
authors.affiliation: Department of Hematology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Otto
authors.initials: O
authors.lastname: Visser
authors.affiliation: Department of Epidemiology and Biostatistics, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Birgit I
authors.initials: BI
authors.lastname: Witte
authors.affiliation: Department of Pathology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Saskia A G M
authors.initials: SA
authors.lastname: Cillessen
authors.affiliation: Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Chris J
authors.initials: CJ
authors.lastname: Mulder
authors.affiliation: Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Gerd
authors.initials: G
authors.lastname: Bouma
mesh_terms: D000328:Adult; D000368:Aged; D002446:Celiac Disease / Q000150:complications / Q000401:mortality / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016399:Lymphoma, T-Cell / Q000150:complications / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D033581:Stem Cell Transplantation; D015996:Survival Rate