Primary abdominal lymphoma

Presenting manifestation of celiac sprue or complicating dermatitis herpetiformis
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      In patients with celiac sprue the risk of having a primary abdominal lymphoma is increased. We have studied the lymphoma aspect of this celiac sprue-lymphoma relationship. During a four year period, seven patients with abdominal lymphoma were found on subsequent investigation to have celiac sprue or the closely linked disorder, dermatitis herpetiformis. Five of the patients had a small intestinal lymphoma, one had a gastric lymphoma and one had a retroperitoneal lymphoma. Histologically, six of the seven tumors were of a diffuse histiocytic type and one was undifferentiated. In four patients the ulcerating small intestinal lymphoma was initially misinterpreted as “benign ulcerative nongranulomatous jejunoileitis,” a condition also reported to complicate celiac sprue. In several patients the associated celiac sprue was clinically occult and could readily have been missed. The celiac sprue was fully responsive in five of the patients who were treated with a gluten-free diet. Our studies suggest that celiac sprue and abdominal lymphoma occur together more often than is currently appreciated. The frequency of this association may be overlooked for two reasons: the associated celiac sprue is sometimes mild and may remain undetected; and, early ulcerative intestinal lymphoma may be mistaken for “benign ulcerative nongranulomatous jejunoileitis.” Patients with abdominal lymphoma should be investigated for celiac sprue and for dermatitis herpetiformis because of the nutritional and pathogenetic implications.
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