Enteropathy‐associated T‐cell lymphoma: A population‐based cohort study on incidence, treatment, and outcome in the Netherlands

eJHaem, vol. 5, pp. 1215–1222

Abstract

Abstract Introduction Enteropathy‐associated T‐cell lymphoma (EATL) is a peripheral T‐cell lymphoma (PTCL) with a poor prognosis. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with or without etoposide consolidated by autologous stem cell transplantation (ASCT) are recommended for fit PTCL patients. The role of etoposide and ASCT in EATL is unclear. Methods This study reports the incidence, treatment, and outcome of EATL patients using the Netherlands Cancer Registry, with nationwide coverage of >95%. Results All patients diagnosed in 1989–2021 (n = 351, 77% treated) were identified (median age 67 years, 56% male, 50% limited stage). Time period analysis assessed trends in primary therapy and overall survival (OS). Treatment included chemotherapy (CT) (34%), surgery (18%), surgery and CT (19%) or CT followed by ASCT (7%). The 5‐year OS for treated patients with limited versus advanced stage was 19% and 9% respectively. The 2‐year OS improved over time (21%–33%, p = 0.06). Surgery only (hazard ratio [HR] 2.16; 95% confidence interval [CI] 1.55–3.01, p < 0.01) and advanced‐stage disease (HR 1.67; 95% CI 1.25–2.23, p = 0.01) were predictors of poor prognosis. ASCT (HR 0.31; 95% CI 0.18–0.56) was associated with improved OS. Conclusion There was no statistical difference in OS between patients treated with or without etoposide. Current first‐line treatment is ineffective.

Authors 14

1. Frederik O. Meeuwes

   University Medical Center Groningen · Medisch Spectrum Twente

   Affiliation as printed

   Department of Hematology Medisch Spectrum Twente Enschede the Netherlands

   Department of Hematology University Medical Center Groningen Groningen the Netherlands

2. Mirian Brink

   University Medical Center Groningen · Netherlands Comprehensive Cancer Organisation

   Affiliation as printed

   Department of Hematology University Medical Center Groningen Groningen the Netherlands

   Department of Research and Development Netherlands Comprehensive Cancer Organization (IKNL) Utrecht the Netherlands

3. Wouter J. Plattel

   Medisch Spectrum Twente

   Affiliation as printed

   Department of Hematology Medisch Spectrum Twente Enschede the Netherlands

4. Joost S.P. Vermaat Aachen Department of Hematology Leiden University Medical Center Leiden The Netherlands

   Leiden University Medical Center

   Affiliation as printed

   Department of Hematology Leiden University Medical Center Leiden the Netherlands

5. Marie José Kersten

   Amsterdam University Medical Centers · Cancer Center Amsterdam · University of Amsterdam

   Affiliation as printed

   Department of Hematology Amsterdam University Medical Center, Cancer Center Amsterdam University of Amsterdam Amsterdam the Netherlands

6. Mariëlle J. Wondergem

   Amsterdam University Medical Centers · Cancer Center Amsterdam · University of Amsterdam

   Affiliation as printed

   Department of Hematology Amsterdam University Medical Center, Cancer Center Amsterdam University of Amsterdam Amsterdam the Netherlands

7. Otto J. Visser

   Isala

   Affiliation as printed

   Department of Hematology Isala Hospital Zwolle the Netherlands

8. Marjolein van der Poel

   Maastricht University Medical Centre · Maastricht University

   Affiliation as printed

   Department of Internal Medicine Division of Hematology GROW School for Oncology and Developmental Biology, Maastricht University Medical Center Maastricht the Netherlands

9. Rimke Oostvogels

   University Medical Center Utrecht

   Affiliation as printed

   Department of Hematology University Medical Center Utrecht Utrecht the Netherlands

10. Feng Jung Sherida H. Woei-A-Jin

    Universitair Ziekenhuis Leuven

    Affiliation as printed

    Department of General Medical Oncology University Hospitals Leuven Leuven Belgium

11. Lara H. Böhmer

    Haga Hospital

    Affiliation as printed

    Department of Hematology Haga Teaching Hospital the Hague the Netherlands

12. Tjeerd J.F. Snijders

    Medisch Spectrum Twente

    Affiliation as printed

    Department of Hematology Medisch Spectrum Twente Enschede the Netherlands

13. Gerwin A Huls

    University Medical Center Groningen

    Affiliation as printed

    Department of Hematology University Medical Center Groningen Groningen the Netherlands

14. Marcel Nijland corresponding

    University Medical Center Groningen

    Affiliation as printed

    Department of Hematology University Medical Center Groningen Groningen the Netherlands