International Journal of Hematology and Oncology 2020, Vol 30, Num 2 Page(s): 057-061
IMMUNOPROLIFERATIVE SMALL BOWEL DISEASE (IPSID): 18 YEARS HACETTEPE EXPERIENCE

HAKAN GÖKER1, GÜLTEN TEKUZMAN1, İBRAHİM BARİSTA1, İBRAHİM GÜLLÜ1, DİNÇER FIRAT1

Hacettepe Üniversitesi Onkoloji Enstitüsü, ANKARA

Keywords: ipsid, chemotherapy, lymphoma
Immunoproliferative Small Intestinal Disease (IPSID), highly prevalent in Mediterranean and Middle -Eastern countries, is a primary extranodal MALT-type lymphoma characterized by dense lymphomatous infiltration of the small intestine and associated with anomalous immunoglobulin alpha-heavy chain secretion, especially in early stages of disease. In this article, we present the treatment and long-term outcome of 26 patients diagnosed with IPSID at Hacettepe University Hospital between Jan 1974 and Sep 1992. The most common presenting symptoms and findings among our patients were: chronic diarrhea (85%), weight loss (%85), abdominal pain (%57), clubbing of the fingers (%31). anemia (%65), elevation of sedimentation rate (%69) and hypoalbuminemia (%84). One patient in early-stage (prelymphomatous) IPSID received 12 months of 1 gm/day oral tetracycline antibiotic therapy and enjoyed long-term, disease-free survival. Among patients who were in later stages (lymphomatous) of IPSID, the majority received CHOP chemotherapy while the remainder received COP; a long-term CR rate of 71% was observed. 10-year estimates of both DPS (disease-free survival) and OAS (overall survival) were 63%. In conclusion, long-term DPS can be achieved with CHOP-like chemotherapy regimens in patients with IPSID. Antibiotic therapy should be considered as the initial treatment approach for patients with early stages of (pre-lymphomatous) disease.