Mezger J, Malfertheiner P, Glasmacher A, Breuer TH, Fischer HP, Meybehm M, Kipnowski HJ, Sauerbruch T.
Medizinische Klinik und Poliklinik-Allgemeine Innere Medizin und Pathologisches Institut, Universitat Bonn sowie Innere Medizin-Rheumatologie, Bruderkrankenhaus St. Petrus, Bonn.
Z Gastroenterol. 1996 Oct;34(10):699-703.
A 22-year-old Libyan patient suffering from chronic diarrhea presented with an alpha-heavy chain paraprotein and a lympho-plasmacellular lymphoma infiltration of the duodenal mucosa. These findings supported the diagnosis of “immunoproliferative small intestinal disease” (IPSID). In this disease, that occurs almost solely in countries with low socioeconomic status, a diffuse infiltration of small intestinal mucosa by neoplastic lymphoid cells causes chronic malabsorption. About 65% of patients exhibit a paraprotein in serum, urine or jejunal juice that consists of the heavy chain of immunoglobulin A (alpha-heavy chain). In advanced stages, IPSID resembles histologically and clinically high grade lymphoma: some patients develop masses in the gut wall, an abdominal lymphadenopathy and involvement of other organs including bone marrow. The disease is believed to be triggered by a chronic infectious antigenic stimulus. Thus, in early stages in some patients cure may be achieved by antibiotic therapy alone. In advanced disease, chemotherapy including anthracyclins is necessary.
Keywords: chronic diarrhea,immunoproliferative small intestinal disease,IPSID