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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.

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  • Authors+Show Affiliations

    ,

    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.

    , ,

    Source

    Blood 119:11 2012 Mar 15 pg 2458-68

    MeSH

    Antineoplastic Combined Chemotherapy Protocols
    Celiac Disease
    Combined Modality Therapy
    Diet, Gluten-Free
    Enteropathy-Associated T-Cell Lymphoma
    HLA-DQ Antigens
    Hematopoietic Stem Cell Transplantation
    Homozygote
    Humans
    Middle Aged
    Prognosis
    Radiotherapy Dosage
    Risk Factors

    Pub Type(s)

    Journal Article
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    22271451

    Citation

    Di Sabatino, Antonio, et al. "How I Treat Enteropathy-associated T-cell Lymphoma." Blood, vol. 119, no. 11, 2012, pp. 2458-68.
    Di Sabatino A, Biagi F, Gobbi PG, et al. How I treat enteropathy-associated T-cell lymphoma. Blood. 2012;119(11):2458-68.
    Di Sabatino, A., Biagi, F., Gobbi, P. G., & Corazza, G. R. (2012). How I treat enteropathy-associated T-cell lymphoma. Blood, 119(11), pp. 2458-68. doi:10.1182/blood-2011-10-385559.
    Di Sabatino A, et al. How I Treat Enteropathy-associated T-cell Lymphoma. Blood. 2012 Mar 15;119(11):2458-68. PubMed PMID: 22271451.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - How I treat enteropathy-associated T-cell lymphoma. AU - Di Sabatino,Antonio, AU - Biagi,Federico, AU - Gobbi,Paolo G, AU - Corazza,Gino R, Y1 - 2012/01/23/ PY - 2012/1/25/entrez PY - 2012/1/25/pubmed PY - 2012/5/23/medline SP - 2458 EP - 68 JF - Blood JO - Blood VL - 119 IS - 11 N2 - 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. SN - 1528-0020 UR - https://www.unboundmedicine.com/medline/citation/22271451/full_citation L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=22271451 DB - PRIME DP - Unbound Medicine ER -