Enteropathy-associated and monomorphic epitheliotropic intestinal t-cell lymphomas
This chapter describes the clinical, pathological, and genetic characteristics of enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma, and refractory celiac disease (RCD), a rare lymphoproliferative disorder that is a precursor to a subset of EATLs. It discusses current and emerging treatment options. Epidemiologic studies indicate a low prevalence of RCD in the community. In contrast to EATL, a female predominance is observed in RCD. Chemotherapy and stem-cell transplantation have been used for refractory RCD II, but these therapies have shown limited benefit with regard to long term disease control and are associated with substantial adverse effects. Future studies need to identify genetic and other risk factors for the progression of RCD to EATL.