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United European Gastroenterol J. 2018 Dec;6(10):1485-1495. doi: 10.1177/2050640618800540. Epub 2018 Sep 12.

Risks for lymphoma and gastrointestinal carcinoma in patients with newly diagnosed adult-onset celiac disease: Consequences for follow-up: Celiac disease, lymphoma and GI carcinoma.

Author information

1
Celiac Center Amsterdam, Department of Gastroenterology and Hepatology, VU University Medical Center, Amsterdam, the Netherlands.
2
Foundation PALGA (The Nationwide Network and Registry of Histo- and Cytopathology in the Netherlands), Houten, the Netherlands.
3
Department of Epidemiology and Biostatistics, the Netherlands Cancer Institute/Antoni van Leeuwenhoek, Amsterdam, the Netherlands.
4
Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands.

Abstract

Background:

The association between celiac disease (CD) and the development of lymphoid and gastrointestinal (GI) malignancies have been reported. However, data are scarce yet needed to develop evidence-based follow-up programs.

Objective:

The objective of this article is to assess relative (RR) and absolute risks of lymphoma and GI carcinoma for newly diagnosed patients.

Methods:

A case-control design to determine RR was performed with cases (lymphoma or GI carcinoma) and controls (melanoma or basal cell carcinoma) diagnosed 1994-2014, retrieved from the Dutch nationwide population-based pathology database (PALGA). Within this population, individuals with histologically proven CD before or simultaneously diagnosed with the malignancy were identified.

Results:

A total of 349/301,425 cases (0.1%) and 282/576,971 (0.05%) controls were diagnosed with CD. Risk of T-cell lymphoma, predominantly enteropathy-associated T-cell lymphoma (EATL), was strongly associated with CD diagnosis (RR = 35.8 (95% CI 27.1-47.4)). Although most often synchronously diagnosed, T-cell lymphoma RR ≥ 1 year after CD diagnosis was still elevated (RR = 12.7 (95% CI 7.6-21.3)). Other CD-associated malignancies were small bowel adenocarcinoma (RR = 11.9 (95% CI 8.2-17.2)) and esophageal squamous cell carcinoma (RR = 3.5 (95% CI 2.1-5.8)). Absolute risks were relatively low. Other types of lymphomas and GI carcinomas were not associated with CD.

Conclusion:

Increased risk for specific malignancies in CD should alert physicians for EATL (both intestinal and extraintestinal) and small bowel adenocarcinoma in patients with CD diagnosed at age ≥ 50 years.

KEYWORDS:

Celiac disease; enteropathy-associated T-cell lymphoma; follow-up; small bowel adenocarcinoma; squamous cell carcinoma

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