{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T16:14:13Z","timestamp":1787847253690,"version":"build-2784847793"},"reference-count":0,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dig Dis"],"published-print":{"date-parts":[[2020]]},"abstract":"<b><i>Background:<\/i><\/b> Refractory celiac disease type II (RCD-II) is a very rare yet severe complication of celiac disease (CD) with a 50% rate of progression to Enteropathy-associated T-cell lymphoma (EATL). Timely diagnosis and treatment of RCD-II is of the essence and requires the identification of a population of frequently clonal, phenotypically aberrant intraepithelial lymphocytes (IELs). Flow Cytometry of intestinal IELs is the recommended method to identify the aberrant surface CD3-negative (sCD3<sup>\u2013<\/sup>) intracytoplasmic CD3-positive (icCD3\u03b5<sup>+<\/sup>) IELs, and a proportion of &#x3e;20% is diagnostic of RCD-II. There is substantial heterogeneity in the clinical course of RCD-II, and insufficient information on prognostic factors. <b><i>Aim:<\/i><\/b> To establish flow cytometric predictors of the clinical evolution of RCD-II, to help guide treatment approaches. <b><i>Patients and Methods:<\/i><\/b> Retrospective single-center study of clinical and immunological features of 6 RCD-II patients and a control group, both identified from a 2,000-patient cohort over 16 years. IEL subset frequencies and the intensity of staining for surface (s) and intracytoplasmic (ic) CD3\u03b5+ on IEL subsets were quantified and correlated with the clinical outcome. <b><i>Results:<\/i><\/b> Unexpectedly, the frequency of aberrant sCD3<sup>\u2013<\/sup> icCD3\u03b5<sup>+<\/sup> cells at diagnosis did not correlate with histological or clinical affection. However, a higher intensity of icCD3\u03b5<sup>+<\/sup> staining in the aberrant IELs relative to expression on normal IELs correlated with monoclonality and with worse clinical outcomes. <b><i>Conclusion:<\/i><\/b> The ratio of icCD3\u03b5<sup>+<\/sup> on aberrant IELs vs. normal IELs appears to be a useful indicator of prognosis at the time of diagnosis, and may represent a novel tool in the follow-up of RCD-II patients after therapy.<\/jats:p>","DOI":"10.1159\/000506305","type":"journal-article","created":{"date-parts":[[2020,2,4]],"date-time":"2020-02-04T22:00:31Z","timestamp":1580853631000},"page":"490-499","source":"Crossref","is-referenced-by-count":12,"title":["The Intracellular Intensity of CD3 on Aberrant Intraepithelial Lymphocytes Is a Prognostic Factor of the Progression to Overt Lymphoma in Refractory Celiac Disease Type II (Pre-Enteropathy-Associated T Cell Lymphoma)"],"prefix":"10.1159","volume":"38","author":[{"given":"Carlota","family":"Garc\u00eda-Hoz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Crespo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Lopez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"De\u00a0Andr\u00e9s","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8986-0311","authenticated-orcid":false,"given":"Raquel","family":"R\u00edos\u00a0Le\u00f3n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Almudena","family":"Sant\u00f3n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Garriga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Butz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"Le\u00f3n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garbi\u00f1e","family":"Roy\u00a0Ari\u00f1o","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2020,2,4]]},"container-title":["Digestive Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/506305","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/506305","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:49:08Z","timestamp":1745423348000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000506305"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020]]},"references-count":0,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2020,11,6]]},"published-print":{"date-parts":[[2020,11,10]]}},"URL":"https:\/\/doi.org\/10.1159\/000506305","archive":["Portico"],"relation":{},"ISSN":["0257-2753","1421-9875"],"issn-type":[{"value":"0257-2753","type":"print"},{"value":"1421-9875","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020]]}}}