Aims: Five cases of primary gastrointestinal (GI) lymphoma (three in t
he stomach, one in the ileum (IPSID) and one in the colon) associated
with localized AL amyloidosis were studied to identify morphological o
r immunohistochemical features which could explain the amyloid deposit
ion. Methods and results: All the cases were low-grade marginal zone B
-cell lymphomas; one case of gastric lymphoma and the IPSID also had a
high-grade component. The lymphomas had a monoclonal plasma cell popu
lation, with different light and heavy-chain type expression in the fi
ve cases, Plasma cell differentiation was closely associated with the
amyloid deposits. The latter were an incidental microscopic finding in
one case, but produced tumoral masses in the others.Conclusions: The
presence of amyloid in primary GI lymphoma is rare, but can have diagn
ostic value. In the present study, neither particular features of the
lymphomatous proliferation nor specific agents are identified. Therefo
re, the factors predisposing to amyloid deposition require elucidation
.