Brief Article
Copyright ©2011 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Jan 28, 2011; 17(4): 506-513
Published online Jan 28, 2011. doi: 10.3748/wjg.v17.i4.506
Evaluation of Cladribine treatment in refractory celiac disease type II
Greetje J Tack, Wieke HM Verbeek, Abdul Al-Toma, Dirk J Kuik, Marco WJ Schreurs, Otto Visser, Chris JJ Mulder
Greetje J Tack, Wieke HM Verbeek, Department of Gastroenterology and Hepatology, VU University Medical Center, 1007 MB Amsterdam, The Netherlands
Abdul Al-Toma, Department of Gastroenterology and Hepatology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands
Dirk J Kuik, Department of Epidemiology and Biostatistics, VU University Medical Center, 1007 MB Amsterdam, The Netherlands
Marco WJ Schreurs, Department of Pathology, VU University Medical Center, 1007 MB Amsterdam, The Netherlands
Otto Visser, Department of Haematology, VU University Medical Center, 1007 MB Amsterdam, The Netherlands
Chris JJ Mulder, Department of Gastroenterology and Hepatology, VU University Medical Center, 1007 MB Amsterdam, The Netherlands
Author contributions: Tack GJ, Al-Toma A and Mulder CJJ designed the research; Tack GJ and Verbeek WHM performed the research; Kuik DJ contributed statistical analytic tools; Tack GJ and Verbeek WHM analyzed the data and wrote the paper; Mulder CJJ, Visser O and Schreurs MWJ drafted the article and revised it critically for important intellectual content.
Supported by An Unrestricted Grant from Astra Zeneca
Correspondence to: Greetje J Tack, MD, Department of Gastroenterology and Hepatology, VU University Medical Center, PO Box 7057, 1007 MB Amsterdam, The Netherlands. g.tack@vumc.nl
Telephone: +31-20-4440613 Fax: +31-20-4440554
Received: June 8, 2010
Revised: September 27, 2010
Accepted: October 3, 2010
Published online: January 28, 2011
Abstract

AIM: 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.

CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.

Keywords: Cladribine; Refractory celiac disease; Clinical course; Enteropathy associated T-cell lymphoma; Survival