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Abnormal intestinal intraepithelial lymphocytes in refractory sprue
C Cellier1, N Patey, L Mauvieux
1Department of Gastroenterology, Hôpital Laënnec, Paris, France.
Gastroenterology
|March 13, 1998
Summary
Refractory sprue involves abnormal intestinal lymphocytes lacking T-cell receptors but expressing CD3epsilon. These cells show restricted T-cell receptor gamma gene rearrangements, offering insights into refractory sprue pathogenesis.
Area of Science:
- Gastroenterology
- Immunology
- Celiac Disease Research
Background:
- The exact cause of refractory sprue remains unknown.
- Understanding its pathogenesis is crucial for patient management.
- This study investigates intestinal lymphocytes in refractory sprue patients.
Purpose of the Study:
- To analyze the phenotype and T-cell receptor (TCR) gene rearrangement status of intestinal lymphocytes.
- To gain insight into the pathogenesis of refractory sprue.
- To compare these features in patients with refractory sprue, active celiac disease, and healthy controls.
Main Methods:
- Intestinal biopsy specimens were analyzed from 26 adults (6 refractory sprue, 7 active celiac disease, 13 controls).
- Immunohistochemistry and flow cytometry were used to study lymphocyte phenotype.
- TCR gene rearrangements were assessed using TCRgammaV-J junctional regions.
Main Results:
- Refractory sprue patients exhibited massive infiltration of intestinal epithelium by specific lymphocytes.
- These lymphocytes lacked CD8, CD4, and surface TCR, but had intracytoplasmic CD3epsilon.
- Restricted TCRgamma gene rearrangements were observed in refractory sprue lymphocytes.
Conclusions:
- Refractory sprue is linked to an aberrant intraepithelial lymphocyte subset.
- These abnormal lymphocytes express CD3epsilon and have TCRgamma chain rearrangements.
- The lack of surface T-cell receptors on these cells is a key finding.
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