title: 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 patients 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.
authors.affiliation: Department of Medical Oncology, Ibn-i Sina Hospital, Ankara University School of Medicine, Sihhiye-Ankara, Turkey.
authors.forename: H
authors.initials: H
authors.lastname: Akbulut
authors.forename: I
authors.initials: I
authors.lastname: Soykan
authors.forename: F
authors.initials: F
authors.lastname: Yakaryilmaz
authors.forename: F
authors.initials: F
authors.lastname: Icii
authors.forename: F
authors.initials: F
authors.lastname: Aksoy
authors.forename: S
authors.initials: S
authors.lastname: Haznedaroglu
authors.forename: S
authors.initials: S
authors.lastname: Yildirim
mesh_terms: D000293:Adolescent; D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007161:Immunoproliferative Small Intestinal Disease / Q000188:drug therapy* / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000008:administration & dosage; D011344:Procarbazine / Q000008:administration & dosage; D013752:Tetracycline / Q000008:administration & dosage*; D016896:Treatment Outcome; D014421:Turkey; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000900:Anti-Bacterial Agents; D011344:Procarbazine; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D013752:Tetracycline; D011241:Prednisone