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Am J Med. 2003 Aug 15;115(3):191-5

Risk of malignancy in patients with celiac disease.

Green PH, Fleischauer AT, Bhagat G, Goyal R, Jabri B, Neugut AI.

Departments of Medicine (PHRG, RG, AIN), College of Physicians and
Surgeons, New York, New York, USA

Studies from Europe have demonstrated an increased risk of malignancy,
especially non-Hodgkin's lymphoma, in patients with celiac disease.
However, there are no data on the risk for similar patients in the United
States. Our aim was to estimate the risk of malignancy in a cohort of
patients with celiac disease compared with the general U.S. population and
to determine if a gluten-free diet is protective.Patients with celiac
disease seen between July 1981 and January 2000 at a referral center were
included. Standardized morbidity ratios (SMRs) (ratio of observed to
expected) and corresponding 95% confidence intervals (CI) were calculated,
using data from the National Cancer Institute's Surveillance, Epidemiology,
and End Results Program. Forty-three (11%) of 381 celiac disease patients
had a diagnosis of cancer; 9 were after the diagnosis of celiac disease, 7
were simultaneous (during same month or admission), and 27 were before the
diagnosis. The standardized morbidity ratio for all cancers combined was
1.5 (95% CI: 0.3 to 7.5), with significantly increased values for small
bowel cancer (SMR = 34; 95% CI: 24 to 42), esophageal cancer (SMR = 12; 95%
CI: 6.5 to 21), non-Hodgkin's lymphoma (SMR = 9.1; 95% CI: 4.7 to 13), and
melanoma (SMR = 5.0; 95% CI: 2.1 to 12). Following the diagnosis of celiac
disease, patients were at increased risk of non-Hodgkin's lymphoma only
(SMR = 6.2; 95% CI: 2.9 to 14), despite adherence to a gluten-free diet.
The non-Hodgkin's lymphoma included both T-cell and B-cell types and
occurred in both gastrointestinal (n = 5) and extraintestinal sites (n =
4).In this cohort of patients with celiac disease, we observed increased
risks of small intestinal adenocarcinoma, esophageal cancer, melanoma, and
non-Hodgkin's lymphoma. The risk of non-Hodgkin's lymphoma persisted
despite a gluten-free diet.

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