@article {Verkarre205, author = {V Verkarre and V Asnafi and T Lecomte and N Patey Mariaud-De Serre and M Leborgne and E Grosdidier and C Le Bihan and E Macintyre and C Cellier and N Cerf-Bensussan and N Brousse}, title = {Refractory coeliac sprue is a diffuse gastrointestinal disease}, volume = {52}, number = {2}, pages = {205--211}, year = {2003}, doi = {10.1136/gut.52.2.205}, publisher = {BMJ Publishing Group}, 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 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.}, issn = {0017-5749}, URL = {https://gut.bmj.com/content/52/2/205}, eprint = {https://gut.bmj.com/content/52/2/205.full.pdf}, journal = {Gut} }