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Immunoproliferative small intestinal disease associated
with Campylobacter jejuni
Marc Lecuit
Service des Maladies, Infectieuses et Tropicales,, Hôpital
Necker-Enfants Malades, Université Paris 5, Paris,
France
Background. Immunoproliferative small intestinal disease (IPSID,
also known as alpha-chain disease) is a form of lymphoma that
arises in small intestinal mucosa-associated lymphoid-tissue
(MALT), and is associated with the expression of a monotypic
truncated immunoglobulin a-heavy chain without associated light
chain. Early stage IPSID responds to antibiotics, suggesting a
bacterial origin. Previous attempts to identify a causative agent
have failed.
Methods. Polymerase chain reaction (PCR), DNA sequencing,
fluorescence in situ hybridization (FISH) and immunohistochemical
studies were performed on intestinal biopsies obtained from a
series of patients with IPSID.
Results. Analysis of frozen intestinal tissue obtained from an
index patient with IPSID who exhibited a dramatic response to
antibiotics revealed the presence of Campylobacter jejuni. A
follow-up retrospective analysis of archival intestinal biopsies
disclosed Campylobacter spp. in four of six additional patients
with IPSID.
Conclusions. These results indicate an association between
Campylobacter spp. and IPSID, and suggest that C. jejuni should be
considered a candidate for addition to the growing list of human
pathogens responsible for immunoproliferative states.[>Read full article in PDF]