CELIAC-ASSOCIATED LYMPHOMA - A SINGLE INSTITUTION EXPERIENCE OF 30 CASES IN THE COMBINATION CHEMOTHERAPY ERA

Celiac sprue (CS) is frequently complicated by malignancy, most common ly small intestinal lymphoma. Our study was performed in an area with a high prevalence of CS to define the clinical features, response to t reatment, and outcome of this tumor. Of a total of 31 lymphomas compli cating CS identified, 30 case records and 24 tumor specimens were revi ewed. Overall 1-year survival was 9 of 29 (31%) and 5-year survival 3 of 27 (11%). Seven previously diagnosed celiac patients developed lymp homa; length an gluten-free diet ranged from 12 to 252 months (median 44 months), In this group, presentation was nonspecific, diagnosis dif ficult, and survival poor (lymphoma diagnosed in life in four of seven , mean survival 2.25 months), Twenty-three patients had CS and lymphom a diagnosed during the same illness. In this group, 14 of 23 presented with a surgical emergency and were treated with tumor resection and c hemotherapy. Nine are disease-free and alive or died of another cause after 10-196 months (mean 74 follow-up). Celiac-associated lymphoma is a frequent, difficult to diagnose, and commonly fatal complication of CS. An aggressive diagnostic approach, including laparoscopy, is reco mmended. Long-term survival can be expected in a significant number of these patients and in our series was almost exclusively confined to t hose treated with chemotherapy.