A 22-year-old Libyan patient suffering from chronic diarrhea presented
with an alpha-heavy chain paraprotein and a lymphoplasmacellular lymp
homa 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 soc
ioeconomic status, a diffuse infiltration of small intestinal mucosa b
y 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 abdo
minal lymphadenopathy and involvement of other organs including bone m
arrow. The disease is believed to be triggered by a chronic infectious
antigenic stimulus. Thus, in early stages in some patients cure may b
e achieved by antibiotic therapy alone. In advanced disease, chemother
apy including anthracyclins is necessary.