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Enteropathy-Associated T-Cell Lymphoma

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Hematopathology

Part of the book series: Encyclopedia of Pathology ((EP))

Synonyms

Enteropathy-type T-cell lymphoma; Epitheliotropic intestinal T-cell lymphoma

Definition

Enteropathy-associated T-cell lymphoma (EATL) is a lymphoma arising from peripheral gut-associated CD103 and CD8 expressing T cells. These cells can either be T-cell receptor (TCR) αβ or TCR γδ expressing T cells. The lymphoma is associated with enteropathy that either precedes lymphoma development or is concurrent with it. Two types are currently recognized. EATL type I now designated as EATL is associated with celiac disease and consists of large pleomorphic lymphoma cells. EATL type II now, designated as Monomorphic Epitheliotropic Intestinal T-cell Lymphoma (MEITL) has no known association with food intolerance and consists of medium-sized monomorphic lymphoma cells. The cause of MEITL is not known.

EATL, as celiac disease, is highly associated with HLA-DQ2 or HLA-DQ8 haplotype. EATL may be preceded by therapy-resistant celiac disease. Refractory celiac disease is diagnosed when the...

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References and Further Reading

  • Chan, J. K., Chan, A. C., Cheuk, W., et al. (2011). Type II enteropathy-associated T-cell lymphoma: A distinct aggressive lymphoma with frequent γδ T-cell receptor expression. American Journal of Surgical Pathology, 35, 1557–1569.

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  • Chott, A., Haedicke, W., Mosberger, I., et al. (1998). Most CD56+ intestinal lymphomas are CD8 + CD5-T-cell lymphomas of monomorphic small to medium size histology. American Journal of Pathology, 153, 1483–1490.

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  • Delabie, J., Holte, H., Vose, J. M., et al. (2011). Enteropathy-associated T-cell lymphoma: Clinical and histological findings from the international peripheral T-cell lymphoma project. Blood, 118, 148–155.

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  • Jantunen, E., Boumendil, A., Finel, H., Lymphoma Working Party of the EBMT, et al. (2013). Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: A retrospective study by the EBMT. Blood, 121, 2529–2532.

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  • Malamut, G., Chandesris, O., Verkarre, V., et al. (2013). Enteropathy associated T cell lymphoma in celiac disease: A large retrospective study. Digestive and Liver Disease, 45, 377–384.

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  • Sharaiha, R. Z., Lebwohl, B., Reimers, L., et al. (2012). Increasing incidence of enteropathy-associated T-cell lymphoma in the United States, 1973–2008. Cancer, 118, 3786–3792.

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  • Tse, E., Gill, H., Loong, F., Kim, S. J., et al. (2012). Type II enteropathy-associated T-cell lymphoma: A multicenter analysis from the Asia Lymphoma Study Group. American Journal of Hematology, 87, 663–668.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Laboratory Medicine and Pathobiology, University Health Network and University of Toronto, Toronto, ON, Canada

    Jan M. A. Delabie

Authors
  1. Jan M. A. Delabie
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Correspondence to Jan M. A. Delabie .

Editor information

Editors and Affiliations

  1. Department of Pathology, Paris Descartes University, University of Paris, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Thierry J. Molina

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Delabie, J.M.A. (2020). Enteropathy-Associated T-Cell Lymphoma. In: Molina, T.J. (eds) Hematopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-95309-0_1970

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  • DOI: https://doi.org/10.1007/978-3-319-95309-0_1970

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-95308-3

  • Online ISBN: 978-3-319-95309-0

  • eBook Packages: MedicineReference Module Medicine

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