title: Evaluation of Cladribine treatment in refractory celiac disease type II.
abstract: OBJECTIVE
  To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II.
  
  
  METHODS
  An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-cell lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated.
  
  
  RESULTS
  Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died.
  
  
  CONCLUSIONS
  Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
authors.affiliation: Department of Gastroenterology and Hepatology, VU University Medical Center, Amsterdam, The Netherlands. g.tack@vumc.nl
authors.forename: Greetje J
authors.initials: GJ
authors.lastname: Tack
authors.forename: Wieke H M
authors.initials: WH
authors.lastname: Verbeek
authors.forename: Abdul
authors.initials: A
authors.lastname: Al-Toma
authors.forename: Dirk J
authors.initials: DJ
authors.lastname: Kuik
authors.forename: Marco W J
authors.initials: MW
authors.lastname: Schreurs
authors.forename: Otto
authors.initials: O
authors.lastname: Visser
authors.forename: Chris J J
authors.initials: CJ
authors.lastname: Mulder
mesh_terms: D000328:Adult; D000368:Aged; D002446:Celiac Disease / Q000188:drug therapy* / Q000473:pathology* / Q000503:physiopathology*; D017338:Cladribine / Q000627:therapeutic use*; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012074:Remission Induction
chemical_list: D007166:Immunosuppressive Agents; D017338:Cladribine