An aberrant immunophenotype and monoclonality of intraepithelial lymphocytes are frequently found in refractory celiac disease.
However, the utility of continual monitoring of intraepithelial lymphocyte immunophenotype and clonality in the surveillance of refractory coeliac disease remains to be studied.
Dr Liu and colleagues from the United Kingdom evluated diagnostic and follow-up biopsies from 33 patients with celiac disease, 7 with suspected refractory celiac disease, 41 with refractory celiac disease, and 20 with enteropathy-associated T cell lymphoma.
The team investigated the biopsiesby CD3/CD8 double immunohistochemistry, and PCR-based clonality analysis of the rearranged T cell receptor genes.
|The aberrant immunophenotype was found in 73%|
The researchers ocaasionally detected an aberrant immunophenotype and monoclonality in celiac disease biopsies.
These abnormalities were found either transiently in patients with celiac disease not compliant with a gluten-free diet or in those who subsequently developed suspected refractory celiac disease, refractory celiac disease or enteropathy-associated T cell lymphoma.
In contrast, the aberrant immunophenotype and monoclonality were found in 73% and 65% biopsies, respectively, at the time of refractory celiac disease diagnosis.
Among the patients with refractory coeliac disease who did not show these abnormalities in their diagnostic biopsies, 80% and 45% of cases gained an aberrant immunophenotype and monoclonality, respectively, during follow-up.
Irrespective of whether detected in diagnostic or follow-up biopsies, the research team found that persistence of both abnormalities was characteristic of refractory coeliac disease.
Importantly, the presence of concurrent persistent monoclonality and aberrant immunophenotype, especially =80% CD3?+CD8- intraepithelial lymphocytes, was a strong predictor of enteropathy-associated T cell lymphoma development in patients with refractory celiac disease.
Dr Liu's team concluded, “Continual monitoring of both immunophenotype and clonality of intraepithelial lymphocytes is more important than snapshot analysis for refractory celiac disease diagnosis and follow-up."
"It could provide a useful tool for surveillance of patients at risk of enteropathy-associated T cell lymphoma."