Celiac Disease - NIH Consensus Development Program - National ...

patients, EATL is disseminated at diagnosis and the outlook is poor, though it may improve withchemotherapy and surgery. The 1-year survival has been estimated to be 30 percent while the5-year survival is approximately 10 percent. (5)Different studies have shown a significant association between adenocarcinoma of thesmall intestine and CD. In the largest series of this rare tumour, an associated CD was found in13 percent of cases, CD being diagnosed first in 63 percent of cases (mean lag time of 8.2 years).CD-associated adenocarcinoma was characterized by proximal (duodeno/jejunal) localizationand presentation with either acute (obstruction, haemorrhage) or chronic signs (anemia,abdominal pain, weight loss). In the majority of patients, complete surgical resection waspossible, and the overall survival at 30 months was 58 percent. (3)In a population-based cohort study from Sweden, an increased risk of oropharingeal(standardized incidence ratio = SIR of 2.3) and esophageal (SIR = 4.2) carcinomas was found inpatients with CD on long-term followup. This work also disclosed a minimal increase of the riskfor colorectal cancer (SIR = 1.5) which was owing to an increased risk for ascending andtransverse but not descending colon or rectal cancer. (6)The Magnitude of the Cancer RiskPioneer studies suggested that the CD-associated relative risk (RR) of developing NHL orother malignancies was very high, in the range of 40–100. This issue has recently beenreconsidered in the light of significant advances on CD epidemiology and diagnosis.To quantify the risk for developing NHL of any primary site associated with CD, weperformed a case control study on 653 Italian adults with NHL at the onset using the serum IgAclass antiendomysial antibody as the screening test. CD was diagnosed in only 6 of 653 patients(0.92 percent) with lymphoma. Of the six cases, three were of B-cell and three were of T-cellorigin. Four of six cases had lymphoma primarily located in the gut. The odds ratio (adjusted forage and sex) for NHL of any primary site associated with CD was 3.1 (95 percent CI 1.3–7.6),16.9 for gut lymphoma (95 percent CI 7.4–38.7), and 19.2 for T-cell lymphoma (95 percent CI7.9–46.6). (7)The Swedish cohort study found a CD-associated overall SIR of 5.9 for malignantlymphoma (95 percent CI 4.3–7.9). The excess occurrence of malignant lymphoma was confinedto adults, decreased with time of followup evaluation, and decreased over successive calendarperiods. The analysis of the causes of death in the same cohort of CD patients showed thatmortality from all malignant neoplasm combined was elevated 70 percent and was particularlyhigh for cancer of the small intestine, NHL, and liver. (6)In summary, these new studies seem to indicate that (1) CD is associated with asignificantly increased risk for NHL, especially of the T-cell type and primarily localized in thegut (EATL), and other GI cancers; (2) the CD–lymphoma association is less common thanpreviously thought, the RR being much lower than 10; and (3) the CD–cancer association ispartially responsible for the increased mortality observed in patients with CD.70