Abstract | BACKGROUND: Refractory coeliac sprue (RCS) with an immunophenotypically aberrant clonal intraepithelial lymphocyte (IEL) population is considered a cryptic form of intestinal T cell lymphoma. AIMS: To investigate the distribution of the abnormal and monoclonal IEL population in the digestive tract of RCS patients. PATIENTS AND METHODS: We compared the frequency of lymphocytic gastritis (LG) and lymphocytic colitis (LC), together with IEL phenotype and T cell clonality, in gastric and colonic samples from 15 adults with RCS (all with aberrant CD3 intracytoplasmic(+) surface(-) CD8(-) clonal IELs on duodenojejunal biopsies), 18 patients with active coeliac disease (ACD), and 10 patients with coeliac disease (CD) on a gluten free diet (GFD-CD) by means of immunohistochemistry and multiplex polymerase chain reaction amplification of the T cell receptor gamma gene (TCR-gamma) rearrangement. Blood samples of nine RCS patients were also tested for clonality. RESULTS: LG was found in 9/14 (64%), 11/18 (61%), and 3/10 (30%) patients with RCS, ACD, and GFD-CD, respectively, while LC was found in 6/11 (55%), 3/4 (75%), and 2/3 (66%) patients. Contrary to CD, all samples from patients with LG and LC showed an aberrant IEL phenotype. Monoclonal TCR-gamma rearrangements were detected in 8/13 (62%), 8/10 (80%), and 4/9 (44%) of gastric, colonic, and blood samples, respectively, from RCS patients, while in CD patients such rearrangements were only found in 2/25 (8%) gastric samples. CONCLUSION: The immunophenotypically aberrant monoclonal IEL population present in the small intestine of patients with RCS frequently disseminates to the blood and the entire gastrointestinal epithelium, suggesting that this is a diffuse gastrointestinal disease.
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Authors | V Verkarre, V Asnafi, T Lecomte, N Patey Mariaud-de Serre, M Leborgne, E Grosdidier, C Le Bihan, E Macintyre, C Cellier, N Cerf-Bensussan, N Brousse |
Journal | Gut
(Gut)
Vol. 52
Issue 2
Pg. 205-11
(Feb 2003)
ISSN: 0017-5749 [Print] England |
PMID | 12524401
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Antibodies, Monoclonal
- Antigens, CD
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Topics |
- Adult
- Aged
- Antibodies, Monoclonal
(immunology)
- Antigens, CD
(immunology)
- Celiac Disease
(immunology)
- Colitis
(immunology)
- Colon
(immunology)
- Female
- Gastric Mucosa
(immunology)
- Gastritis
(immunology)
- Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
(immunology)
- Humans
- Intestinal Mucosa
(immunology)
- Lymphocyte Count
- Male
- Middle Aged
- Phenotype
- Polymerase Chain Reaction
(methods)
- Prospective Studies
- T-Lymphocytes
(immunology)
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