Small bowel malignant lymphoma complicating celiac sprue and the mesenteric lymph node cavitation syndrome

Malignant small intestinal lymphoma may complicate or antedate clinical recognition of celiac sprue. However, histologic diagnosis of lymphoma is made especially difficult in the presence of small bowel ulceration. A 70-yr-old man with celiac sprue and a history of dermatitis herpetiformis was initially seen for recurrent diarrhea; panmalabsorption with steatorrhea and protein-losing enteropathy were documented. Subsequent studies showed ectopic gastric mucosa in the small bowel, hyposplenism with mesenteric lymph node cavitation, and small bowel erosions and ulceration. Despite strong clinical suspicion for more than 2 yr, only 1 of 88 small bowel biopsy specimens was positive for lymphoma. At autopsy, shortly after histologic diagnosis of lymphoma, extensive small bowel involvement and infiltration were observed. This is the first report of lymphoma complicating the recently described nonneoplastic lymphoreticular syndrome associated with celiac sprue characterized by splenic atrophy and mesenteric lymph node cavitation.