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Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience
  1. Abdulbaqi Al-toma
  1. VU University Medical Center, Netherlands
    1. Wieke Verbeek
    1. VU University Medical Center, Netherlands
      1. Mohammed Hadithi
      1. VU University Medical Center, Netherlands
        1. Mary von Blomberg
        1. VU University Medical Center, Netherlands
          1. Chris Mulder (cjmulder{at}
          1. VU University Medical Center, Netherlands


            Background: Coeliac disease may be regarded as refractory disease (RCD) when symptoms persist or recur despite strict adherence to a gluten free diet. RCD may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-cell population, respectively. RCD I seems to respond well to azathioprine/prednisone therapy. RCD II is usually resistant to any known therapy and transition into Enteropathy-Associated T-cell Lymphoma (EATL) is common.

            Aim: to provide further insight into RCD and the development of EATL, by reporting on long term survival, risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease.

            Design and Methods: Retrospective comparison of responses to therapy in four groups of patients with complicated coeliac disease: 43 RCD-I, 50 RCD II (total), of whom 26 RCD II who developed EATL after a period of refractoriness to a gluten free diet (secondary EATL) and 13 EATL patients without preceding history of complicated coeliac disease (de novo EATL).

            Results: No coeliac disease related mortality is recognized in the RCD I group. The overall five year survival in RCD I is 96%, in RCD II (total) is 58% and in RCD II after developing EATL is only 8%. The 2 year survival in the de novo EATL is 20% versus 15% in secondary EATL (P=0.63).Twenty eight (56%) from 50 patients with RCD-II died, 23 (46%) due to EATL and 4 due to progressive refractory state with emaciation and one from neurocoeliac disease.

            Conclusion: Remarkably, no patient with RCD I developed RCD II or EATL within mean follow up of five years (range 2-15 years). Fifty two percent of the RCD II patients developed EATL within 4-6 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.

            • EATL
            • Refractory coeliac disease
            • mortality
            • prognosis
            • stem cell transplantation

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