How I treat How I treat enteropathy-associated T-cell lymphoma

(EATL) is a complication of celiac disease (CD). This tumor derives from the neoplas-tic transformation of aberrant intraepithe-lial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagno-sis of CD are recognized as risk factors for malignant evolution of CD. Recur-rence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance en-teroclysis, positron emission tomogra-phy scan, and histologic identification of lesions represent the best options. Treat-ment includes high-dose chemotherapy preceded by surgical resection and fol-lowed by autologous stem cell transplan-tation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL. (Blood. 2012;119(11): 2458-2468