Lymphoma & related disorders
Mature T/NK cell disorders
Enteropathy associated

Topic Completed: 1 August 2011

Minor changes: 12 January 2021

Copyright: 2001-2021,, Inc.

PubMed search: enteropathy associated T cell lymphoma [title]

Dragos C. Luca, M.D.
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Cite this page: Luca DC. Enteropathy associated. website. Accessed January 17th, 2021.
Definition / general
  • Enteropathy associated T cell lymphoma (EATL) is an intestinal tumor of intraepithelial T lymphocytes showing varying degrees of transformation but usually presenting as a tumor composed of large, often polymorphic lymphoid cells, which are negative for CD56
  • Also called intestinal T cell lymphoma (with or without enteropathy), but many other T cell lymphomas can present with intestinal involvement
ICD coding
  • ICD-O: 9717 / 3 - intestinal T cell lymphoma
  • Rare aggressive disease associated with gluten sensitive enteropathy (celiac disease)
  • Higher frequency in areas with high prevalence of celiac disease (northern Europe)
  • Monomorphic variant (type II EATL): 10% - 20% of cases of EATL, broader geographic distribution and sporadic occurrence in areas where celiac disease is rare (Asia)
  • Lymphomas of small intestine may derive from various subsets of intestinal intraepithelial lymphocytes that are differentially activated by diverse antigenic stimuli
  • Association with celiac disease: positive serology, HLA DQ2 or DQ8 expression, dermatitis herpetiformis, hyposplenism
  • Monomorphic variant NOT associated with celiac disease, may represent a distinct entity
  • EATL in situ: refractory celiac disease with intraepithelial lymphocytes showing immunophenotypic and genetic features similar to EATL
  • Recent studies evaluating the risk of lymphoma in patients with celiac disease: Table
  • Typically affects jejunum or ileum (ulcers with possible perforation)
  • May occur in duodenum, stomach, colon or outside the GI tract, but rare
Clinical features
  • Childhood onset celiac disease in a minority of patients
  • Adult onset celiac disease or celiac disease diagnosed simultaneously with lymphoma in most patients
  • Abdominal pain, often intestinal perforation, sometimes prodrome of refractory celiac disease accompanied by ulceration (ulcerative jejunitis - UJ)
Prognostic factors
Treatment and Prognosis:
  • Usually poor for both forms, with recurrences most frequently in the small intestine and death from abdominal complications superimposed on uncontrolled malabsorption
  • Proposed management to avoid perforation (Case Rep Oncol 2009;2:36)
Case reports
Postulated normal counterpart
  • Intraepithelial T cells of the intestine
Gross description
  • Multiple ulcerated mucosal masses, also single / multiple ulcers or large exophytic mass
Gross images

Images hosted on other servers:

Well defined mural mass

Resected small
intestine with
multiple patchy
segmented lesions

Microscopic (histologic) description
  • Variably sized cells with abundant intraepithelial T cells, often infiltrating the individual crypt epithelium; villous atrophy due to celiac disease may be present
  • Most commonly: relatively monotonous medium to large cells with round / angulated vesicular nuclei, prominent nucleoli and moderate / abundant pale cytoplasm
  • Less commonly: marked pleomorphism with multinucleated cells similar to anaplastic large cell lymphoma
  • Infiltration by inflammatory cells with numerous histiocytes and eosinophils, sometimes obscuring the lymphoma
  • Enteropathy in the adjacent mucosa: villous atrophy, crypt hyperplasia, lamina propria lymphoplasmacytic infiltrate, intraepithelial lymphocytosis
  • Monomorphic variant (type II EATL): medium sized, round, dark nuclei, rim of pale cytoplasm, florid crypt intraepithelial infiltration, prominent intraepithelial lymphocytosis in the adjacent mucosa, no inflammatory background, less necrosis
Microscopic (histologic) images

Images hosted on other servers:

Various images

Positive stains
  • CD3, CD7, CD103, cytotoxic proteins TIA1, perforin, granzyme B
  • Variable CD8 and TCRβ
  • CD30 in a variable proportion of tumor cells but in almost all cases
  • Monomorphic variant: CD3+, CD4-, CD8+, CD56+, TCRβ+
Negative stains
  • CD4, CD5, CD56
Molecular / cytogenetics description
  • Clonal rearrangement of TRB@ and TRG@ genes
  • HLADQA1*0501, DQB1*0201 genotype (celiac disease)
  • Complex segmental amplifications of the 9q31.3-qter region or del16q12.1 (58% - 70% of cases, both forms)
  • Classical form: +1q, +5q
  • Monomorphic form: 8q24 (MYC) amplifications
  • TCR gene rearrangements studies for EATL (Fig.2) and UJ (Fig.3) (Am J Pathol 1997;151:493)
Differential diagnosis
  • B cell lymphoma, histiocytic neoplasms, anaplastic carcinoma, melanoma
Additional references
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