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Gut. 2003 Feb;52(2):205-11.

Refractory coeliac sprue is a diffuse gastrointestinal disease.

Author information

  • 1Department of Pathology and Université René Descartes-Paris V (EA219), AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France. virginie.verkarre@nck.ap-hop-paris.fr

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.

Comment in

PMID:
12524401
[PubMed - indexed for MEDLINE]
PMCID:
PMC1774980
Free PMC Article

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