RESULTS: All
IPSID patients presented with the clinical and laboratory features of severe intestinal malabsorption, and all had diffuse lymphoplasmacytic infiltration in the mucosa of the small bowel.
Lymphomas were localized mainly in the jejunum and mesenteric nodes. The histologic subtypes were diffuse large cell in two, immunoblastic in three, and diffuse mixed in one. All patients responded poorly to
chemotherapy, with a median survival duration of 10.5 months. The common presenting symptoms of the 39 non-
IPSID patients included
abdominal pain (90%),
weight loss (31%), abdominal mass (26%), obstruction (26%), and perforation (23%). Diffuse large-cell and immunoblastic
lymphomas constituted 82% of cases. Four patients had stage IE, 19 stage II 1E, and 16 stage 112E disease according to the Musshoff's criteria; 22 had bulky
tumors and 19 had multiple
tumors. The
tumors were completely resected in 14 patients. Of 33 patients treated with
combination chemotherapy, 73% achieved a complete remission. With a median follow-up duration of 90 months, there have been four relapses, with only one at the primary
tumor site. The overall 5-year survival and disease-free survival rates for non-
IPSID patients who were treated with
chemotherapy were 59% and 54%, respectively.
CONCLUSION: