Cavitation of mesenteric lymph nodes, splenic atrophy, and a flat small intestinal mucosa. Report of six cases.

Abstract

This study describes, in 6 patients with a flat small intestinal mucosa and splenic atrophy, a particular lesion of the mesenteric lymph nodes termed "cavitation." In 4 women and 2 men with abdominal mass, intestinal obstruction, or suspected celiac disease-associated lymphoma, unusual pseudocystic lymph node lesions were found in the jejunal or jejunoileal mesentery. These lesions consisted histologically of a large central cavity occupied by hyaline-type material and surrounded by fibrous tissue and remnants of lymph node structures. There was no histologic evidence of malignant lymphoma or mesenteric panniculitis. Diffuse subtotal villous atrophy involving at least the jejunum was found in each case, together with unequivocal biological and morphological evidence of splenic atrophy, severe malabsorption, and a history of chronic or childhood diarrhea. HLA B8 or DR3, or both, was present in 4 of 4 cases; dermatitis herpetiformis was present in 1 case. An unequivocal mucosal response to a gluten-free diet was observed in 2 cases. Four patients died of cachexia or hyposplenism-related infections. We conclude that cavitation of mesenteric lymph nodes is an original feature which may be associated with splenic atrophy and a flat small intestinal mucosa; some of these patients may have celiac disease. Pathogenesis is unknown.