5-YEAR RESULTS OF THE TREATMENT OF 23 PATIENTS WITH IMMUNOPROLIFERATIVE SMALL-INTESTINAL DISEASE - A TURKISH EXPERIENCE

BACKGROUND, Currently, there is no agreement regarding optimal treatme nt strategies for immunoproliferative small intestinal disease (IPSID) . In this article, the authors report the treatment outcomes of a grou p of 23 Turkish patients with IPSID. METHODS, Between December 1988 an d July 1993, 23 consecutive patients with IPSID, including 5 with secr etory type, were included in the study. Seven patients with Stage A di sease (according to the criteria of Galien et al.) received tetracycli ne (1 g/day, orally) for a median duration of 7 months (range, 6-11 mo nths) initially, whereas the remaining patients (9 Stage B patients an d 7 Stage C patients) received combination chemotherapy (cyclophospham ide, vincristine, procarbazine, and prednisolone [COPP regimen]) follo wed 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 medi an follow-up was 68 months (range, 38-89 months). As first line therap y in Stage A patients, tetracycline yielded a 71% CR and 43% disease f ree 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 (GAS) rate for the entire group was 70%, and the 5-year DFS rate for patien ts with CR was 75%. However, the median OAS for 3 patients with immuno blastic lymphoma was only 7 months. CONCLUSIONS. The COPP regimen, wit h 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 ly mphoma whereas tetracycline appears to be the initial treatment of cho ice for patients with Stage A disease. (C) 1997 American Cancer Societ y.