IMM UNOPROLIFER ATI VE SMALL INTES TINAL DISEASE (ALPHA HEAVY CHAIN DISEASE) : UNUSU AL PRESENTATION WI TH ABDOMINAL MASS

During the period of 1984-1995, nine young patients were admitted to Shiraz University Hospitals with a chief complaint of abdominal mass, weight loss and chronic diarrhea. Clinically, these cases were diagnosed as intestinal tuberculosis or lymphoma. Laparotomy revealed a huge mass involving the upper small intestine. Histologic sections showed diffuse infiltration of the intestinal wall with numerous mature and immature plasma cells. Serological tests showed increased levels of IgA. These cases were diagnosed and treated as immunoproliferative small intestinal disease (IPSID). Follow-up of these patients revealed that seven patients died 6-8 months after the operation and the other two patients were lost to follow-up.