title: How I treat enteropathy-associated T-cell lymphoma.
abstract: Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence 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 enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL.
authors.affiliation: First Department of Medicine, Centro per lo Studio e la Cura della Malattia Celiaca, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo, University of Pavia, Pavia, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Di Sabatino
authors.forename: Federico
authors.initials: F
authors.lastname: Biagi
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Gobbi
authors.forename: Gino R
authors.initials: GR
authors.lastname: Corazza
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002446:Celiac Disease / Q000209:etiology* / Q000628:therapy*; D003131:Combined Modality Therapy; D055050:Diet, Gluten-Free; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D006683:HLA-DQ Antigens / Q000378:metabolism; D018380:Hematopoietic Stem Cell Transplantation; D006720:Homozygote; D006801:Humans; D008875:Middle Aged; D011379:Prognosis; D011879:Radiotherapy Dosage; D012307:Risk Factors
chemical_list: D006683:HLA-DQ Antigens; C053186:HLA-DQ2 antigen