Enteropathy-Associated T-cell Lymphoma | Molecular Pathology Laboratory Network, Inc.

Enteropathy-Associated T-cell Lymphoma

enteropathy associated T-cell lymphoma malignant lymphocytes

The majority of Enteropathy-associated T-cell lymphomas (EATL) are associated with adult onset gluten-sensitive enteropathy with which they have a similar geographic distribution, which are usually CD56- CD103+. A minor subset of EATLs (Type II; monomorphic variant) is not as clearly associated with gluten-sensitive enteropathy. These subsets are typically CD56+ CD8+. Both subtypes are CD4-, and >80% positive for chromosomal gains at chromosome 9q31.3. In the case of Type II EATL, clinical correlation is required to exclude unlikely secondary GI involvement by an unusual CD56+ peripheral T-cell lymphoma, NOS.

Many adult patients with clinically severe celiac disease that is refractory to a gluten-free diet harbor aberrant clonal intestinal T-cells with an EATL-like immunophentype, suggesting an intermediate stage of evolution between gluten-sensitive enteropathy and enteropathy-associated T-cell lymphoma.

History
Microscopy
References

A 72 year-old male with no known history of gluten-sensitive enteropathy underwent segmental small bowel resection for intestinal perforation.

Routine histologic sections demonstrate extensively ulcerated intestinal mucosa that is largely effaced by sheets of medium sized, relatively monomorphic malignant lymphocytes with round to slightly irregular, vesicular nuclei and focal infiltration into muscle wall. By paraffin immunohistochemistry, tumor cells are CD3+ T-cells, uniformly positive for CD3 and CD7 with aberrant loss of CD5. They are uniformly negative for CD20, CD4 and CD30; most are positive for CD8 and CD56. No atrophic benign intestinal mucosa epithelium is identified.