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Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience
  1. A Al-toma
  1. VU University Medical Center, Netherlands
    1. W H. M Verbeek
    1. VU University Medical Center, Netherlands
      1. M Hadithi
      1. VU University Medical Center, Netherlands
        1. B M. E von Blomberg
        1. VU University Medical Center, Netherlands
          1. C J. J Mulder (cjmulder{at}vumc.nl)
          1. VU University Medical Center, Netherlands

            Abstract

            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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