Enteropathy-associated T-cell lymphoma
Classification and external resources
ICD-O:	M9717/3

Enteropathy-Associated T-cell Lymphoma (EATL) is a T-cell lymphoma of the small intestine. It is the most common primary gastrointestinal T-cell lymphoma, arising from the T cells that are found between the cells that line the small intestinal (brush border cells or small intestinal epithelial cells).^[1] These cancerous T-cells are a consequence of prolonged, untreated coeliac disease in genetically susceptible individuals.

[edit] Genetics

Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Triticeae glutens (e.g wheat gluten). In gluten-sensitive individuals with EATL, 68% are homozygotes of the DQB1*02 subtype at the HLA-DQB1 locus .^[2] (See Coeliac Disease, HLA-DQ, HLA DR3-DQ2) A DQ isoform that appears to be responsible for EATL in the overwhelming number of cases is highly effective at presenting a proteolytically protected region of α2-glaidin to T-cells, constant over-stimulation of T-cell eventually results in neoplastic growth.^[3] EATL typically appears after the 4th decade of life, within 3 years of coeliac disease diagnosis or in undiagnosed coeliacs.^[4][5] In treated coeliacs, EATL may be preceded by refractory coeliac disease 1(RCD1) or, prominently, refractory celiac disease 2 (RCD2), in which EATL is a frequent outcome^[6] Refractory coeliac disease is no longer favorably responsive to wheat-gluten abstinence. Beyond the RCD1 stage, many drugs are not effective, and undetected coeliac disease leading to de novo EATL generally has a poor outcome.

Early recognition of coeliac disease, particularly with a focus on DQ2 homozygotes and in affected family members, is the only effective prevention, though bone marrow transplant was suggested as a treatment during early RCD2.^[7]

[edit] See also

• Coeliac Disease
• Lymphoma
• MALT

[edit] References

v • d • e Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208) Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590-9739, 9800-9839) B cell (lymphoma, leukemia) (most CD19, CD20) By development/ marker TdT+ ALL (Precursor B acute lymphoblastic leukemia/lymphoma) CD5+ naive B cell (CLL/SLL) mantle zone (Mantle cell) CD22+ Prolymphocytic · CD11c (Hairy cell leukemia) CD79a+ germinal center/follicular B cell (Follicular, Burkitt's, GCB-DLBCL) marginal zone/marginal-zone B cell (Splenic marginal zone, MALT, Nodal marginal zone) RS (CD15+,CD30+) Classic Hodgkin's lymphoma (Nodular sclerosis) · CD20 (Nodular lymphocyte predominant Hodgkin's lymphoma) PCDs/PP (CD38+/CD138+) see immunoproliferative immunoglobulin disorders By infection KSHV (Primary effusion) · EBV (Lymphomatoid granulomatosis, Post-transplant lymphoproliferative disorder) · HIV (AIDS-related lymphoma) · Helicobacter pylori (MALT lymphoma) T/NK T cell (lymphoma, leukemia) (most CD3, CD4, CD8) By development/ marker TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma) prolymphocyte (Prolymphocytic) CD30+ (Anaplastic large cell, Lymphomatoid papulosis) By location/peripheral Cutaneous (Mycosis fungoides, Sézary's disease) · Hepatosplenic · Angioimmunoblastic · Enteropathy-associated T-cell lymphoma By infection HTLV-1 (Adult T-cell leukemia/lymphoma) NK cell/ (most CD56) Aggressive NK-cell leukemia · Blastic NK cell lymphoma T or NK EBV (Extranodal NK-T-cell lymphoma) · Large granular lymphocytic leukemia Lymphoid+myeloid Acute biphenotypic leukaemia Lymphocytosis Lymphoproliferative disorders (X-linked lymphoproliferative disease, Autoimmune lymphoproliferative syndrome) · Leukemoid reaction · Pseudolymphoma lymphocyte navs: cells/physio, immunodeficiency/immunoproliferative immunoglobulin/neoplasia, proc

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