Small bowel (small intestine) - Enteropathy-associated T cell lymphoma (EATL)

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Small bowel (small intestine)

Lymphoma

Enteropathy-associated T cell lymphoma (EATL)

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Intestinal tumour of intraepithelial T lymphocytes composed of large, often polymorphic lymphoid cells, which are negative for CD56 (WHO)
● Associated with long standing malabsorption (mean 28 years) that ceases to be gluten-sensitive
● 14% incidence in celiac sprue patients
● Abdominal pain, clubbing, fever, hemorrhage, intestinal obstruction, perforation, ulceration of bowel, weight loss; also sustained increase in serum IgA
● Ulceration may coincide with or precede detectable lymphoma, but it is difficult to rule out lymphoma (unless have multiple biopsies) since it may be focal
● 75% dead within 2 years

Gross description
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● Widespread patchy involvement, often of small bowel, associated with ulceration, stricture, perforation

Micro description
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● May require 20 tissue sections to diagnose malignancy since tumor is so focal

WHO classification:

Type I (celiac associated):
● Variable lymphomatous infiltrate with neoplastic large cells with conspicuous nucleoli
● May have pleomorphic, multinucleated cells
● Also small to medium-sized lymphocytes, histiocytes and eosinophils
● Adjacent mucosa shows villous atrophy, crypt hyperplasia, increased inflammatory cells in lamina propria, intraepithelial lymphocytosis

Type II (no history of celiac sprue):
● Lymphomatous infiltrate is homogeneous and composed of small to medium-sized lymphocytes with a rim of pale or clear cytoplasm, slightly irregular nuclei and small nucleoli; CD8+, CD56+
● Villous atrophy, blunting and intraepithelial lymphocytosis confined to area of lymphoma
● More distant nonlymphomatous mucosa is unremarkable

Micro images
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Various images

Positive stains
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● CD3, CD7, CD30

Negative stains
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● CD4, CD8, CD22, CD68

Additional references
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● Arch Pathol Lab Med 2011;135:1283

End of Small bowel (small intestine) > Lymphoma > Enteropathy-associated T cell lymphoma (EATL)

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