DIFFUSE LYMPHOPLASMACYTIC INFILTRATION OF THE SMALL-INTESTINE WITH DAMAGE TO NERVE PLEXUS - A CAUSE OF INTESTINAL PSEUDOOBSTRUCTION

We describe the clinicopathologic characteristics of three patients wi th chronic intestinal pseudo-obstruction and malabsorption. The patien ts were young women (average age, 25 years) who presented with abdomin al pain, nausea, vomiting, diarrhea, and weight loss that led to extre me inanition and death in two patients despite multiple treatment sche mes. The evolution of the process averaged 8 years. No case manifested evidence of malignant lymphoproliferative progression. Histologically , a diffuse lymphoplasmacytic infiltrate that affected all the layers of the intestinal wall was observed in full-thickness biopsy specimens . The proliferating lymphocytes were small and mixed with mature plasm a cells that proved to be polyclonal on immunohistochemical analysis. An outstanding finding in all three cases was extensive damage to subm ucosal and myenteric nerve plexus associated with a lymphoid infiltrat e. Quantification of the myenteric plexus by using immunohistochemical and morphometric techniques also revealed a marked reduction in their number. We concluded that diffuse lymphoplasmacytic infiltration of t he small intestine associated with damage to the intestinal nerve plex us constitutes a specific disorder that is different from other diseas es that produce intestinal pseudo-obstruction.