ABSTRACT

A small subgroup of adult celiac disease patients may be resistant to a gluten free diet (GFD); these patients are classified as refractory celiac disease (RCD). Depending on the immunophenotyping of small bowel intraepithelial lymphocytes (IEL), there are two subdivisions of RCD. Type-1 RCD has a normal IEL phenotype; while type-II RCD is a severe enteropathy with a clonal expansion of abnormal IEL characterized by expression of intracellular CD3 and lack of surface markers CD3, CD8 and the T-cell receptor. RCD-II is pre-lymphoma due to the high probability of developing enteropathy associated T-cell lymphoma (EATL). Autologous hematopoietic stem cell transplantation (autologous HSCT) provides a feasible therapeutic strategy for RCD-II resulting in an impressive improvement, both clinically and biochemically, along with improvement in the overall quality-of-life.