Enteropathy-associated T-cell Lymphoma

Enteropathy-associated T-cell Lymphoma

Sa A. Wang, MD

Enteropathy-associated T-cell lymphoma destroying mucosa and infiltrating into muscle wall of jejunum. Nonneoplastic epithelium is also present image.

Enteropathy-associated T-cell lymphoma, type 1, involving jejunum. Lymphoma cells are large and pleomorphic. Numerous eosinophils and some neutrophils are present in the background.



  • Enteropathy-associated T-cell lymphoma (EATL)


  • Intestinal T-cell lymphoma


  • T-cell lymphoma of intestine arising from intraepithelial T cells


Classical Variant EATL (EATL Type 1)

  • Type 1 comprises 80-90% of cases of EATL

  • These tumors are associated with celiac disease

    • Serological tests (+); association with HLA DQ2 or HLA DQ8

    • Pathologic evidence of celiac disease in uninvolved intestinal mucosa

    • Associated clinical findings: Gluten intolerance, dermatitis herpetiformis, and hyposplenism

  • Refractory celiac disease (RCD) is precursor lesion

    • Definition

      • Persistent enteropathy-associated histologic changes on biopsy despite strict gluten-free diet for > 12 months or

      • Severe persistent symptoms necessitating clinical intervention independent of duration of gluten-free diet

    • In some cases of RCD, intraepithelial lymphocytes show

      • Immunophenotypic aberrancy

      • Monoclonal T-cell receptor gene rearrangement

      • Gain of chromosome 1q

      • These cases may be considered as EATL in situ

Monomorphic Variant (EATL Type 2)

  • Association with celiac disease or other risk factors is not proven

  • 10-20% of cases



  • Incidence

    • Annual rate of 0.5-1 per 1,000,000 people in Western countries

      • Greatest frequency in areas with high prevalence of celiac disease, particularly Northern Europe

      • Accounts for ˜ 35% of all small intestinal lymphomas

      • < 5% of all gastrointestinal tract lymphomas

  • Age

    • Median: 60 years

  • Gender

    • Affects males and females equally


  • EATL most commonly arises in jejunum or ileum

    • Often involves mesenteric lymph nodes

  • Presentation in duodenum, stomach, colon may occur rarely

    • Lymphomas arising in these sites show genetic alterations similar to classic EATL in jejunum or ileum

    • RCD can involve duodenum, stomach, or colon

  • Patients usually do not have peripheral lymphadenopathy

  • EATL can disseminate to liver, bone marrow, spleen, skin, and other organs


  • Clinical history of celiac disease

    • Small proportion of patients have childhood-onset celiac disease

    • Most patients have adult-onset celiac disease

    • Typical symptoms of celiac disease

      • Diarrhea with bulky, foul-smelling, floating stools

      • Malabsorption: Anemia, weight loss, vitamin deficiency

      • Dermatitis herpetiformis

    • In some patients, celiac disease and EATL are diagnosed at same time

    • Most patients with monomorphic variant of EATL do not have clinical history of celiac disease

  • Clinical findings at time EATL is initially diagnosed

    • Most patients have weight loss, abdominal pain, and diarrhea

      • Nonspecific; overlaps with celiac disease

    • Some patients can have B symptoms

    • Substantial subset of patients present with small bowel obstruction or perforation

      • In many of these patients, diagnosis of lymphoma is made by laparotomy

    • In patients with known celiac disease, lymphoma occurs ˜ 5-10 years after celiac disease diagnosis

      • Often heralded by clinical relapse after a period of good response to gluten withdrawal

      • In some patients, lymphoma develops in course of progressive deterioration of RCD

    • Presentation of patients with monomorphic EATL is similar, except there is no history of celiac disease

Endoscopic Findings

  • Lymphoma

    • Multiple ulcerating raised mucosal masses or

    • 1 or more larger ulcers or

    • Large exophytic mass

  • Mucosal changes related to RCD

    • Scalloping, fissuring, loss of folds, or mosaic pattern

Laboratory Tests

  • Celiac disease

    • Serology

      • IgA anti-tissue transglutaminase and IgA endomysial antibody are most sensitive and specific tests

      • Anti-gliadin antibody tests have low sensitivity and specificity, no longer used routinely

    • HLA typing

      • For DQ2 and DQ8 may be useful in individuals with equivocal findings


Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Enteropathy-associated T-cell Lymphoma

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