Cladribine Therapy in Refractory Celiac Disease With Aberrant T Cells

摘要: Background & Aims: Refractory celiac disease (RCD) may be subdivided into RCD types I and II with phenotypically normal aberrant intraepithelial T-cell populations, respectively. In II, transition enteropathy-associated lymphoma (EATL) is seen frequently. We have evaluated the effect of cladribine (2-CDA), a purine analogue inducing depletion, on clinical, histopathologic, immunologic parameters, as well toxicity side effects in group patients. Methods: Between 2000 2005, 17 patients were included (8 men, 9 women). All had clonal rearrangement receptor γ gene immunophenotyping showed an population lacking surface expression CD3, CD8, αβ, presence CD103 intracytoplasmic CD3. Treatment consisted 2-CDA (0.1 mg/kg/day) intravenously for 5 days, given 1–3 courses every 6 months depending response. Results: tolerated without serious effects. Six (35.8%) clinical improvement (weight gain, diarrhea, hypoalbuminemia). 10 (58.8%) significant histologic (35.2%) decrease T cells was seen. Seven (41.1%) developed EATL died subsequently. One patient progressive refractory state emaciation. Conclusions: feasible, tolerated, can induce subgroup patients, albeit it does not prevent development. However, earlier reported potential risk precipitating overt should taken consideration.