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Original Article
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Volume 350:239-248 January 15, 2004 Number 3
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Immunoproliferative Small Intestinal Disease Associated with Campylobacter jejuni
Marc Lecuit, M.D., Ph.D., Eric Abachin, Ph.D., Antoine Martin, M.D., Claire Poyart, M.D., Ph.D., Philippe Pochart, Ph.D., Felipe Suarez, M.D., Djaouida Bengoufa, M.D., Ph.D., Jean Feuillard, M.D., Ph.D., Anne Lavergne, M.D., Jeffrey I. Gordon, M.D., Patrick Berche, M.D., Ph.D., Loïc Guillevin, M.D., and Olivier Lortholary, M.D., Ph.D.

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ABSTRACT

Background Immunoproliferative small intestinal disease (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 {alpha} heavy chain without an associated light chain. Early-stage disease responds to antibiotics, suggesting a bacterial origin. We attempted to identify a causative agent.

Methods We performed polymerase chain reaction (PCR), DNA sequencing, fluorescence in situ hybridization, and immunohistochemical studies on intestinal-biopsy specimens from a series of patients with immunoproliferative small intestinal disease.

Results Analysis of frozen intestinal tissue obtained from an index patient with immunoproliferative small intestinal disease who had a dramatic response to antibiotics revealed the presence of Campylobacter jejuni. A follow-up retrospective analysis of archival intestinal-biopsy specimens disclosed campylobacter species in four of six additional patients with immunoproliferative small intestinal disease.

Conclusions These results indicate that campylobacter and immunoproliferative small intestinal disease are associated and that C. jejuni should be added to the growing list of human pathogens responsible for immunoproliferative states.


Source Information

From Hôpital Avicenne (M.L., A.M., F.S., J.F., L.G., O.L.), Institut Pasteur (M.L., O.L.), Hôpital Necker (E.A., C.P., P.B.), Conservatoire National des Arts et Métiers (P.P.), Hôpital Saint-Louis (D.B.), and Hôpital Lariboisière (A.L.) — all in Paris; and Washington University School of Medicine, St. Louis (J.I.G.).

Address reprint requests to Dr. Lecuit at the Service des Maladies Infectieuses et Tropicales, Hôpital Necker–Enfants Malades, Université Paris V, 149 rue de Sèvres, 75743 Paris CEDEX 15, France, or at mlecuit{at}pasteur.fr.

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Related Letters:

Immunoproliferative Small Intestinal Disease Associated with Campylobacter jejuni
Peterson M. C., Lecuit M., Suarez F., Lortholary O.
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N Engl J Med 2004; 350:1685-1686, Apr 15, 2004. Correspondence

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