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Clinical Trial
. 2011 Jan 28;17(4):506-13.
doi: 10.3748/wjg.v17.i4.506.

Evaluation of Cladribine treatment in refractory celiac disease type II

Greetje J Tack  1 , Wieke H M Verbeek, Abdul Al-Toma, Dirk J Kuik, Marco W J Schreurs, Otto Visser, Chris J J Mulder
Affiliations
Clinical Trial

Evaluation of Cladribine treatment in refractory celiac disease type II

Greetje J Tack et al. World J Gastroenterol. .

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; Clinical course; Enteropathy associated T-cell lymphoma; Refractory celiac disease; Survival.

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Figures

Figure 1
Figure 1
Schematic representation of intracellular pathways involved in 2-CdA cytotoxicity. (Adapted with permission from Borak, 2006). 2-CdA-MP: Cladribine monophosphate; 2-CdA-DP: Cladribine diphosphate; 2-CdA-TP: Cladribine triphosphate; dCK: Deoxycytidine kinase; 5’-NT: 5’-nucleotidase; hENT: Human equilibrative nucleoside transporter; hCNT: Human concentrative nucleoside transporter; AIF: Apoptosis inducing factor; APAF-1: Apoptotic protease activating factor; PARP: Poly (ADP-ribose) polymerase.
Figure 2
Figure 2
Flowchart of the response to cladribine treatment. RCD: Refractory celiac disease; 2-CdA: 2-chlorodeoxyadenosine; auSCT: autologous hematopoietic stem cell transplantation; EATL: Enteropathy associated T-cell lymphoma.
Figure 3
Figure 3
Kaplan-Meier survival curves. A: Survival rate of responders and non-responders to cladribine treatment (Log Rank: P = 0.037). 13 patients died in the 1st and 5th year; 21 patient died in the 2nd, 3rd and 4th year of follow-up; B: Survival rate of the pre-treatment (I) and the upfront 2-chlorodeoxyadenosine (II) group (Log Rank: P = 0.23). 14 patients died in the 1st year; 21 patient died in the 1st, 3rd and 4th year; 32 patients died in the 2nd year of follow-up.
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