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

Abstract

Introduction: Enteropathy associated T-cell lymphoma (EATL) is a rare peripheral T-cell lymphoma (PTCL) associated with celiac disease that has a poor prognosis. Treatment of EATL is challenging due to the lack of clinical trials. In PTCL, the preferred chemotherapeutic regimen is cyclophosphamide, doxorubicine, vincristine and prednisone (CHOP), either with or without etoposide (CHOEP) followed by consolidative autologous stem cell transplantation (ASCT). This nationwide Dutch study describes the incidence, treatment and outcome of all reported EATL cases. Methods: All patients diagnosed with EATL between 1989 and 2020 were identified in the Netherlands Cancer Registry with survival follow-up through February 1, 2022, thereby excluding post-mortem diagnoses. Baseline characteristics, treatment modality and survival outcomes were collected. Patients were categorized into three periods (1989–1999, 2000–2010, 2011–2020) and in limited (I/II) or advanced (III/IV) stage disease. For patients diagnosed as of 2014, detailed information on treatment regimens was available. Overall survival (OS) was defined as the time from diagnosis to all-cause-death. Multivariable analysis of OS was performed using Cox regression. Results: We included 338 patients (median age 67 years [range 37–90 years], 57% male) in our study. Limited stage disease was more common than advanced stage (51% versus 38%, 11% undetermined). Treatment consisted of surgery (18%), chemotherapy (CT; 33%), CT followed by ASCT (3%), surgery and CT (19%), surgery and CT followed by ASCT (3%), surgery and RT (1%) or no treatment (23%). Between 2014 and 2020, the most commonly used CT regimens were CHOP (54% in limited stage and 66% in advanced stage disease) and CHOEP (32% in all stages). Over time, the use of CT increased from 22% to 46%, whereas resection only and resection combined with CT became uncommon (decrease from 33% to 7%, and 23% to 5% respectively; Figure 1). Two-year OS improved from 19% in 1989–1999 to 26% in 2011–2020. The 6-month, 2-year and 5-year OS for limited stage were 51%, 24% and 15% (median OS 6.3 months) and for advanced stage 44%, 16% and 7% (median OS 5.1 months), respectively (Figure 2). In multivariable analysis, surgery only (hazard ratio [HR] 1.97; 95% confidence interval [CI] 1.41–2.74), no therapy (HR 3.66; 95% CI: 1.72–3.76) and advanced stage disease (HR 1.31; 95% CI: 1.02–1.70) were independent predictors of poor prognosis, whereas ASCT (HR 0.38; 95% CI: 0.17–0.81) was associated with improved OS. Keyword: aggressive T-cell non-Hodgkin lymphoma No conflicts of interests pertinent to the abstract.