Small bowel (small intestine) - Mediterranean lymphoma

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Small bowel (small intestine)

Lymphoma

Mediterranean lymphoma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 22 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 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)

Clinical features
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● 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)

Case reports
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● 53 year old woman with immunoproliferative small intestinal disease (Turk J Gastroenterol 2012;23:91)

Gross description
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● Low grade: non flattened mucosa
● High grade: diffusely thickened folds with small nodules in distal duodenum / proximal jejunum

Micro description
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Low grade:
● Heavy lymphoplasmacytic infiltrate by mature cells

High grade:
● Resembles diffuse large cell lymphoma with plasmacytoid features
● May have starry sky pattern, follicular hyperplasia

Micro images
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Various images

Positive stains
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● 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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