Small bowel (small intestine)
Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.
● Also called immunoproliferative small intestinal disease, Middle Eastern lymphoma
● More common among non-European Jews, Middle Eastern Arabs, South African blacks
● B cell lymphoma that arises in background of chronic diffuse mucosal plasmacytosis, with IgA heavy chain synthesis (no variable chain synthesized)
● Many have pre-existing malabsorption
● May initially respond to antibiotics
● Often due to Campylobacter jejuni, another human pathogens responsible for immunoproliferative states (N Engl J Med 2004;350:239)
● 53 year old woman with immunoproliferative small intestinal disease (Turk J Gastroenterol 2012;23:91)
● Low grade: non flattened mucosa
● High grade: diffusely thickened folds with small nodules in distal duodenum / proximal jejunum
● Heavy lymphoplasmacytic infiltrate by mature cells
● Resembles diffuse large cell lymphoma with plasmacytoid features
● May have starry sky pattern, follicular hyperplasia
● Monoclonal kappa or lambda (not both), syndecan1, IgA heavy chain
● BCL6 and p53 expression associated with advanced stage and aggressive behavior (World J Gastroenterol 2006;12:3602)
End of Small bowel (small intestine) > Lymphoma > Mediterranean lymphoma
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