PT  - JOURNAL ARTICLE
AU  - V Verkarre
AU  - V Asnafi
AU  - T Lecomte
AU  - N Patey Mariaud-De Serre
AU  - M Leborgne
AU  - E Grosdidier
AU  - C Le Bihan
AU  - E Macintyre
AU  - C Cellier
AU  - N Cerf-Bensussan
AU  - N Brousse
TI  - Refractory coeliac sprue is a diffuse gastrointestinal disease
AID  - 10.1136/gut.52.2.205
DP  - 2003 Feb 01
TA  - Gut
PG  - 205--211
VI  - 52
IP  - 2
4099  - http://gut.bmj.com/content/52/2/205.short
4100  - http://gut.bmj.com/content/52/2/205.full
SO  - Gut2003 Feb 01; 52
AB  - 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 γ gene (TCR-γ) 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-γ 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.