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Hematopoietic cancer including lymphoma in celiac disease according to Marsh criteria 0-3
Örebro University, School of Health and Medical Sciences.
Örebro University, School of Health and Medical Sciences.ORCID iD: 0000-0001-6328-5494
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(English)Manuscript (Other academic)
Abstract [en]

Background: Celiac disease (CD) is associated with an increased risk of lymphoma, but it is unknown if borderline mucosal damage and latent CD are risk factors for lymphoma.Methods: We examined the risk of hematopoietic cancer in a nationwide population–based cohort of 28,800 individuals with biopsy-verified CD (villous atrophy, Marsh 3), 12,663 individuals with small intestinal inflammation (Marsh 1+2), and 3,551 with latent CD (positive antiendomysial, tissue transglutaminase or antigliadin test but normal mucosa on biopsy). The study participants were identified through all pathology departments (n=28) in Sweden and were biopsied in 1969-2006 (median: 1998). Cox regression estimated the hazard ratio (HR) for hematopoietic malignancies.Results: While biopsy-verified CD and intestinal inflammation were both statistically significantly associated with lymphoma (CD: HR = 3.18; 95% CI = 2.63-3.83; inflammation: 1.66; 1.28-2.17), latent CD was not (1.04; 0.44-2.43). CD was associated with both non-Hodgkin’s (NHL) and Hodgkin’s lymphoma (HL) (4.81; 3.81-6.07 and 4.39; 2.59-7.45 respectively). Risk estimates for NHL and HL were lower in inflammation (1.65; 1.15-2.38 and 1.48; 0.60-3.62 respectively) and latent CD (1.79; 0.74-4.34 and 1.08; 0.13-9.00 respectively). No increased risk of lymphoma was seen in children with a small intestinal biopsy. This study found no association between leukemia and small intestinal pathology.Conclusion: CD is associated with an increased risk of lymphoma. This risk increase was also seen in individuals with small intestinal inflammation. Latent CD is not associated with lymphoma of any kind, and positive CD serology alone cannot be used to predict future risk of lymphoma.

Keyword [en]
autoimmune, biopsy, celiac, coeliac, child, cohort study, cancer, hematopoietic, leukemia, lymphoma, pathology
National Category
Pediatrics Gastroenterology and Hepatology Medical and Health Sciences
Research subject
Pediatrics; Epidemiology; Medicine
Identifiers
URN: urn:nbn:se:oru:diva-5222OAI: oai:DiVA.org:oru-5222DiVA, id: diva2:158220
Available from: 2009-01-30 Created: 2009-01-30 Last updated: 2017-10-18Bibliographically approved
In thesis
1. Associated disorders in celiac disease
Open this publication in new window or tab >>Associated disorders in celiac disease
2009 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Background: Celiac disease (CD) is an autoimmune disorder that affects genetically susceptible individuals and is induced by dietary gluten. Treatment consists of a lifelong gluten-free diet. CD is common and affects about 1% of the general population. The classic symptoms include diarrhea and malabsorption, but many patients have only mild symptoms or no symptoms at all. The proportion of individuals presenting with atypical symptoms or discovered only when investigating an associated condition of CD is increasing.

Aims: The aim of this thesis was to investigate the risk of possible associated disorders through Swedish population-based registers. The objective was to gain more information on the consequences of having CD and to identify high risk groups where screening may be considered.

Materials and methods: We used the Swedish hospital discharge register to examine the risk of liver disease, autoimmune heart disease, Addison’s disease and thyroid disorders in a cohort of about 14,000 individuals with CD and an age and sex matched reference population of 70,000 individuals. In the last study we used all regional pathology registers and the cancer registry to examine the risk of hematopoietic cancer, including lymphoma in three different cohorts: I) 28,810 individuals with CD; II) 12,681 individuals with small intestinal mucosal inflammation but without villous atrophy; and III) 3552 individuals with latent CD (a positive serology test for CD with a normal small intestinal biopsy).

Results: CD is statistically significantly associated with an increased risk of liver disease, Addison’s disease, thyroid disease and lymphoma. We also found an increased risk of lymphoma in individuals with small intestinal mucosal inflammation. There was no statistically significant association between autoimmune heart disease or leukemia and CD. Latent CD was not associated with any hematopoietic cancers.

Conclusion: This thesis found a positive association between CD and a number of autoimmune and inflammatory disorders. Clinicians need to have a high awareness of this association and to test for these conditions when symptoms appear.

Place, publisher, year, edition, pages
Örebro: Örebro universitet, 2009. p. 83
Series
Örebro Studies in Medicine, ISSN 1652-4063 ; 27
Keyword
Addison, autoimmune, biopsy, celiac, child, cohort study, cancer, heart, liver, lymphoma, thyroid
National Category
Pediatrics Medical and Health Sciences
Research subject
Pediatrics; Epidemiology; Medicine
Identifiers
urn:nbn:se:oru:diva-5223 (URN)978-91-7668-649-2 (ISBN)
Public defence
2009-02-27, Wilandersalen, Universitetssjukhuset Örebro, Örebro, 10:00 (English)
Opponent
Supervisors
Available from: 2009-02-06 Created: 2009-01-30 Last updated: 2017-10-18Bibliographically approved

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Elfström, PeterMontgomery, Scott M.Ludvigsson, Jonas F.

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