Enteropathy‐associated T‐cell lymphoma: A population‐based cohort study on incidence, treatment, and outcome in the Netherlands
Frederik O. Meeuwes,
Mirian Brink,
Wouter J. Plattel,
Joost S. P. Vermaat,
Marie José Kersten,
Mariëlle Wondergem,
Otto Visser,
Marjolein W. M. van derPoel,
Rimke Oostvogels,
F. J. Sherida H. Woei‐A‐Jin,
Lara Böhmer,
Tjeerd J. F. Snijders,
Gerwin A. Huls,
Marcel Nijland
Affiliations
Frederik O. Meeuwes
Department of Hematology University Medical Center Groningen Groningen the Netherlands
Mirian Brink
Department of Hematology University Medical Center Groningen Groningen the Netherlands
Wouter J. Plattel
Department of Hematology Medisch Spectrum Twente Enschede the Netherlands
Joost S. P. Vermaat
Department of Hematology Leiden University Medical Center Leiden the Netherlands
Marie José Kersten
Department of Hematology Amsterdam University Medical Center, Cancer Center Amsterdam University of Amsterdam Amsterdam the Netherlands
Mariëlle Wondergem
Department of Hematology Amsterdam University Medical Center, Cancer Center Amsterdam University of Amsterdam Amsterdam the Netherlands
Otto Visser
Department of Hematology Isala Hospital Zwolle the Netherlands
Marjolein W. M. van derPoel
Department of Internal Medicine Division of Hematology GROW School for Oncology and Developmental Biology, Maastricht University Medical Center Maastricht the Netherlands
Rimke Oostvogels
Department of Hematology University Medical Center Utrecht Utrecht the Netherlands
F. J. Sherida H. Woei‐A‐Jin
Department of General Medical Oncology University Hospitals Leuven Leuven Belgium
Lara Böhmer
Department of Hematology Haga Teaching Hospital the Hague the Netherlands
Tjeerd J. F. Snijders
Department of Hematology Medisch Spectrum Twente Enschede the Netherlands
Gerwin A. Huls
Department of Hematology University Medical Center Groningen Groningen the Netherlands
Marcel Nijland
Department of Hematology University Medical Center Groningen Groningen the Netherlands
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.
