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World J Clin Oncol. Mar 24, 2022; 13(3): 200-208
Published online Mar 24, 2022. doi: 10.5306/wjco.v13.i3.200
Possible relationship between refractory celiac disease and malignancies
Kaan Demiroren
Kaan Demiroren, Department of Pediatric Gastroenterology, University of Health Sciences, Yuksek Ihtisas Teaching Hospital, Bursa 16000, Turkey
Author contributions: Demiroren K solely contributed to this manuscript.
Conflict-of-interest statement: The author declares that no conflicts of interest, financial or otherwise, exist related to this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Kaan Demiroren, MD, Associate Professor, Department of Pediatric Gastroenterology, University of Health Sciences, Yuksek Ihtisas Teaching Hospital, Mimar Sinan, Yıldirim, Bursa 16000, Turkey.
Received: April 9, 2021
Peer-review started: April 9, 2021
First decision: July 29, 2021
Revised: August 16, 2021
Accepted: March 6, 2022
Article in press: March 6, 2022
Published online: March 24, 2022
Processing time: 349 Days and 0.7 Hours

Celiac disease (CeD) is a chronic autoimmune disorder that is triggered by gluten in genetically susceptible individuals, and that is characterized by CeD-specific antibodies, HLA-DQ2 and/or HLA-DQ8 haplotypes, enteropathy and different clinical pictures related to many organs. Intestinal lymphoma may develop as a result of refractory CeD. If a patient diagnosed with CeD is symptomatic despite a strict gluten-free diet for at least 12 months, and does not improve with severe villous atrophy, refractory CeD can be considered present. The second of the two types of refractory CeD has abnormal monoclonal intraepithelial lymphocytes and can be considered as pre-lymphoma, and the next picture that will emerge is enteropathy-associated T-cell lymphoma. This manuscript addresses "CeD and malignancies" through a review of current literature and guidelines.

Keywords: Refractory celiac disease, Enteropathy-associated T-cell lymphoma, Pre-lymphoma, Low grade lymphoma

Core Tip: Malignancies are among the leading consequence of celiac disease (CeD), and intestinal lymphoma and adenocarcinomas in particular. Enteropathy-associated T-cell lymphoma type 1 has been shown to develop from refractory CeD type 2, while the association of CeD with other cancer types is controversial. Decades of reported studies suggest that a non-delayed diagnosis of CeD and strict adherence to a gluten-free diet significantly reduces the rate of cancer development associated with CeD.