Lymphoma development and survival in refractory coeliac disease type II: Histological response as prognostic factor

Background Refractory coeliac disease type II (RCDII) frequently transforms into an enteropathy‐associated T‐cell lymphoma (EATL) and therefore requires intensive treatment. Current evaluated treatment strategies for RCDII include cladribine (2‐CdA) and autologous stem cell transplantation (auSCT). Objective The purpose of this study was to evaluate long‐term survival and define clear prognostic criteria for EATL development comparing two treatment strategies. Methods A total of 45 patients were retrospectively analysed. All patients received 2‐CdA, after which they were either closely monitored (monotherapy, n  = 30) or a step‐up approach was used including auSCT (step‐up therapy, n  = 15). Results Ten patients (22%) ultimately developed EATL; nine of these had received monotherapy. Absence of histological remission after monotherapy was associated with EATL development ( p  = 0.010). Overall, 20 patients (44%) died with a median survival of 84 months. Overall survival (OS) within the monotherapy group was significantly worse in those without histological remission compared to those with complete histological remission( p  = 0.030). The monotherapy group who achieved complete histological remission showed comparable EATL occurrence and OS as compared to the step‐up therapy group ( p  = 0.80 and p  = 0.14 respectively). Conclusion Histological response is an accurate parameter to evaluate the effect of 2‐CdA therapy and this parameter should be leading in the decisions whether or not to perform a step‐up treatment approach in RCDII.