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

作者：Nijeboer P^*; van Wanrooij R L J; van Gils T; Wierdsma N J; Tack G J; Witte B I; Bontkes H J; Visser O; Mulder C J J; Bouma G

来源：United European Gastroenterology Journal, 2017, 5(2): 208-217.

DOI：10.1177/2050640616646529

• Refractory coeliac disease type II
• coeliac disease
• complicated coeliac disease
• cladribine
• autologous stem cell transplantation
• enteropathy-associated T-cell lymphoma

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.

• 出版日期2017-3