Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: A Turkish experience

Abstract

BACKGROUND. Currently, there is no agreement regarding optimal treatment strategies for immunoproliferative small intestinal disease (IPSID). In this article, the authors report the treatment outcomes of a group of 23 Turkish patients with IPSID. METHODS. Between December 1988 and July 1993, 23 consecutive patients with IPSID, including 5 with secretory type, were included in the study. Seven patients with Stage A disease (according to the criteria of Galien et al.) received tetracycline (1 g/day, orally) for a median duration of 7 months (range, 6-11 months) initially, whereas the remaining patients (9 Stage B patients and 7 Stage C patients) received combination chemotherapy (cyclophosphamide, vincristine, procarbazine, and prednisolone [COPP regimen]) followed by tetracycline at a dose of 1 g/day for 6 more months in patients with complete response (CR) after the COPP regimen. RESULTS. The median follow-up was 68 months (range, 38-89 months). As first-line therapy in Stage A patients, tetracycline yielded a 71% CR and 43% disease free survival (DFS) rate. Eleven of 16 patients (69%) with Stage B or C disease who received the COPP regimen achieved CR and only 2 patients had a recurrence (DFS rate of 56%). The 5-year overall survival (OAS) rate for the entire group was 70%, and the 5-year DFS rate for patterns with CR was 75%. However, the median OAS for 3 patients with immunoblastic lymphoma was only 7 months. CONCLUSIONS. The COPP regimen, with its acceptable toxicity, appears to be a good alternative as a first line treatment for patients with Stage B or C IPSID with low grade lymphoma whereas tetracycline appears to be the initial treatment of choice for patients with Stage A disease.