Conflict of interest: Nothing to report.
Incidence of lymphoproliferative disorders in patients with celiac disease†
Article first published online: 28 MAY 2012
Copyright © 2012 Wiley Periodicals, Inc.
American Journal of Hematology
Volume 87, Issue 8, pages 754–759, August 2012
How to Cite
Leslie, L. A., Lebwohl, B., Neugut, A. I., Gregory Mears, J., Bhagat, G. and Green, P. H.R. (2012), Incidence of lymphoproliferative disorders in patients with celiac disease. Am. J. Hematol., 87: 754–759. doi: 10.1002/ajh.23237
- Issue published online: 17 JUL 2012
- Article first published online: 28 MAY 2012
- Accepted manuscript online: 26 APR 2012 07:42AM EST
- Manuscript Accepted: 13 APR 2012
- Manuscript Revised: 10 APR 2012
- Manuscript Received: 18 NOV 2011
Prior studies describe an increased incidence of lymphoma in celiac disease. However, few studies differentiate among lymphoproliferative disorders (LPDs). Our aim was to determine incidences of LPD subtypes in celiac disease patients, describe patterns of celiac disease presentation in patients who develop LPD, and compare survival in patients with various LPD subtypes. We conducted a retrospective cohort study of adults with biopsy-proven celiac disease seen at a US referral center from 1981 to 2010, identified patients with comorbid LPD, and calculated standardized incidence ratios (SIR) for each LPD subtype. In our cohort of 1,285 patients with celiac disease, there were 40 patients with LPD [SIR = 6.48, 95% confidence interval (CI) = 4.62–8.64] including 33 with non-Hodgkin lymphoma (NHL, SIR = 6.91, 95% CI = 4.26–8.28). The incidences of NHL subtypes including enteropathy-associated T-cell (EATL, n = 12), non-EATL T-cell (SIR = 22.43, 95% CI = 7.08–46.41), diffuse large B-cell (SIR = 5.37, 95% CI = 1.93–10.52), mantle cell (SIR = 32.21, 95% CI = 6.07–78.97), and marginal zone (SIR = 37.17, 11.73–76.89) lymphoma remained significantly elevated when only those diagnosed with celiac before LPD were considered (n = 24, NHL SIR = 4.47, 95% CI = 2.86–6.44). Patients who developed LPD were older at time of celiac disease diagnosis (57.9 ± 15.5 versus 42.5 ± 17.4 years, P < 0.0001) and more likely to present with diarrhea (60.0% versus 39.8% P = 0.016), abdominal pain (17.5% versus 5.5% P = 0.0046), and/or weight loss (12.5% versus 4.0%, P = 0.028). EATL patients had a shorter average survival than non-EATL NHL patients (3.2 versus 15.0 years, P = 0.016). The incidence of LPD is increased in celiac disease patients. Those diagnosed later in life who present with symptoms of malabsorption are more likely to be diagnosed with LPD. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.