Lymphoma & related disorders
Mature T/NK cell disorders
Intestinal
Enteropathy associated
Author: Dragos C. Luca, M.D.
Topic Completed: 1 August 2011
Minor changes: 5 July 2020
Copyright: 2001-2020, PathologyOutlines.com, Inc.
PubMed search: enteropathy associated T cell lymphoma [title]
Table of Contents
Definition / general | Terminology | ICD coding | Epidemiology | Etiology | Sites | Clinical features | Prognostic factors | Case reports | Postulated normal counterpart | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Luca DC. Enteropathy associated. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBenteropathy.html. Accessed August 13th, 2020.
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
Terminology
- 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
Epidemiology
- 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)
Etiology
- 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
Sites
- 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
- 23 year old woman presenting with acute renal failure (World J Gastroenterol 2006;12:2301)
- 36 year old man with NK-like T cell lymphoma involving ileum but without celiac disease (Arch Pathol Lab Med 2003;127:e142)
- 37 year old man with enteropathy but no celiac disease (Hum Pathol 2004;35:639)
- 47 year old man with tumor developing after intestinal diffuse large B cell lymphoma (Am J Surg Pathol 2007;31:476)
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
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
