Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease

Abstract

(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 1 Department of Medical Oncology, Ibn-i Sina combination chemotherapy (cyclophosphamide, vincristine, procarbazine, and Hospital, Ankara University School of Medicine, prednisolone [COPP regimen]) followed by tetracycline at a dose of 1 g/day for 6 Sihhiye-Ankara, Turkey.more months in patients with complete response (CR) after the COPP regimen. 2 Department of Gastroenterology, Ibn-i Sina RESULTS. The median follow-up was 68 months (range, 38-89 months). As first-Hospital, Ankara University School of Medicine, line therapy in Stage A patients, tetracycline yielded a 71% CR and 43% disease Sihhiye-Ankara, Turkey.free survival (DFS) rate. Eleven of 16 patients (69%) with Stage B or C disease who 3 Department of Third Internal Medicine, SSK received the COPP regimen achieved CR and only 2 patients had a recurrence Hospital, Diskapi-Ankara, Turkey.(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

Heavy chain disease

“…Pharmacologic treatment • Akbulut et al [31] reported 5-year treatment results of 23 Turkish patients with IPSID, including five with secretion of ␣ chains. Seven patients had stage A disease and were treated with tetracycline for a median of 7 months, whereas the remaining 16 patients (nine stage B, seven stage C) received combination chemotherapy (cyclophosphamide, vincristine, procarbazine, and prednisolone).…”

Regression of Immunoproliferative Small Intestinal Disease After Eradication of Helicobacter pylori

“…IPSID has been reported from various parts of the world including India [10,11]. Chronic infection results in constant antigenic stimulation which in the presence of malnutrition and genetic predisposition (HLA B12, HLA A9) results in uncontrolled B cell proliferation [12,13].…”

Malassimilationsyndrom

“…Aufgrund der Hypothese, dass ein infektiöses Agens antigener Stimulus der Immunproliferation sein könnte, wird im Stadium A von den meisten Autoren eine antibiotische Therapie mit Tetracyclin, zwischen 1 und 2 g/Tag empfohlen.Im Verlaufe einer solchen Behandlung können Vollremissionen in 33-71% der untersuchten Fälle erreicht werden [2,3,8,12,16].…”

“…Uneinigkeit besteht über die Behandlungsdauer mit Antibiotika,hier schwanken die Angaben zwischen 6 Monaten und 2 Jahren [2,10,16], in Einzelfällen jedoch sogar über insgesamt 5 Jahre [3].…”

“…Die besten Ansprechraten werden unter doxorubicinhaltigen Therapieschemata mit Vollremissionsraten von bis zu 70% berichtet [5,11,12]. Erkrankungsfrei nach 5 Jahren waren in einer Studie 56% der Patienten nach einer Kombinationschemotherapie [2].Die 5-Jahres-Über-lebenszeit behandelter Patienten unter Berücksichtigung aller Krankheitsstadien beträgt bestenfalls 67% [5]. Für den mittel-bis langfristigen Therapieerfolg entscheidend soll dabei eine Kombination Abb.4a, b Laborwerte im zeitlichen Verlauf.…”