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Pathology of Enteropathy Associated T-Cell Lymphoma

A rare type of peripheral T-cell lymphoma that characteristically involves the jejunum or ileum.

 

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The presence of a monoclonal T-cell population in nonlymphomatous enteropathic small intestinal mucosa has been described in Enteropathy associated  T-cell lymphoma , ulcerative jejunitis, and nonresponsive coeliac disease.

Visit: Malabsorption syndrome ; Coeliac Disease ;An approach to evaluation of small intestinal biopsy

Enteropathy associated T-cell lymphoma (EATL) of the small intestine is a well-documented complication of coeliac disease.

It is present in patients with longstanding coeliac disease ( gluten sensitive enteropathy ) but more often follows a short history of adult coeliac disease.

Patients are relatively young adults (30-40yrs).

Lymphoma is suspected when there is a reversal to response to gluten withdrawal and patients complain of fever, weight loss and abdominal pain.

Extensive sampling of the lesion, careful evaluation of the biopsy and a close follow up is advised as often it can be very difficult to make the diagnosis with certainty.

It has been suggested on the basis of previous molecular analysis, and the immunophenotypic findings that the monoclonal intraepithelial lymphocytes in patients with complications of coeliac disease are neoplastic, although they are not cytologically abnormal and they do not form tumour masses.

Ulcerative jejunitis (UJ) and Enteropathy-associated T-cell lymphoma (EATL) are closely related conditions both associated with celiac disease. Benign-appearing inflammatory ulcers are seen in both, which has led to the suggestion that UJ is a manifestation of EATL.

It has been shown that T-cell monoclonality is a feature of the ulcers in both UJ and EATL and the same clone is present in EATL and its associated inflammatory ulcers and in UJ and subsequently developing lymphoma.

Enteropathy-associated T-cell lymphoma is an aggressive disease with poor prognosis but strict adherence to a gluten free diet may prevent its occurrence.

Normal histology of the small intestine for anatomic pathologists

Microscopic features:

- Ulcers associated with sprue-like mucosa. Fissuring ulcers are noted mimicking Crohn's disease.

- Severe villous atrophy and crypt hyperplasia.

- Dense infiltration of the epithelium by lymphocytes.

- Initially there is a mixed cellular infiltrate.

- Gradual increase in atypical large lymphoid cells. Binucleate or multinucleate cells are noted.

- Eventually there is transformation into full blown lymphoma.

- CD3 (+), CD4 (-), CD8 (+/-), UCHL1 (+), TIA-1 (+), CD103 (+).

- Some cases are associated with Epstein-Barr virus.

- If an intestinal lymphoma morphologically looks like an anaplastic large cell lymphoma and is CD30+, it should not be called an anaplastic large cell lymphoma if there are features consistent with enteropathy-type T-cell lymphoma.

 

Further reading:

A rare case of enteropathy-associated T-cell lymphoma presenting as acute renal failure.

Hematological manifestations of celiac disease.

What is the role of celiac disease in enteropathy-type intestinal lymphoma? A retrospective study of nine cases.

Association of celiac disease and intestinal lymphomas and other cancers.

Frequency of clonal intraepithelial T lymphocyte proliferations in enteropathy-type intestinal T cell lymphoma, coeliac disease, and refractory sprue.

Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group.

Are complicated forms of celiac disease cryptic T-cell lymphomas?

Enteropathy associated T-cell lymphomas have a cytotoxic T-cell phenotype. 

Molecular analysis of T-cell clonality in ulcerative jejunitis and enter opathy-associated T-cell lymphoma.

 

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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

 

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