Results of laparotomy in immunoproliferative small intestinal disease

Immunoproliferative small intestinal diseases (IPSID), previously known as Mediterranean Lymphomas, constitute more than 60% of the non-Burkitt's small intestinal lymphomas in Tunisia. A multidisciplinary study of IPSID was undertaken by the Tunisian/French Lymphoma Study Group in 1980 to reach a better understanding of the two subgroups of the disease: secreting IPSID (essentially alpha-chain disease [ACD]); and nonsecreting IPSID (NS-IPSID) (extensive small intestinal lymphomas without gammopathy). The results of initial exploratory laparotomy performed in 38 cases of IPSID (17 ACD and 21 NS-IPSID) are described here, and show notable similarities between the two groups: the extensive pattern of the abnormal cell infiltrate along the major part of the small intestine; frequent and extensive involvement of the mesenteric lymph nodes; the existence of several degrees of severity in small intestinal mesenteric lymph nodes; and other intraabdominal organ involvement. Certain differences also were observed: the relatively high degree of tropism of the NS-IPSID towards the gastric mucosa which was absent in the ACD of this series; and the more frequent involvement of the entire length of the small bowel in ACD. In spite of these discrete differences, the IPSID form a homogeneous group which is definitely distinguishable from the segmentary small intestinal lymphomas known as Western-type, and which account for approximately one third of our non-Burkitt's small bowel lymphomas.