14 August 2008 – Case of the Week #127
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We thank Drs. Elaine Alt and Marise McNeeley, Quest Diagnostics, New Jersey (USA) for contributing this case. To contribute a Case of the Week, email [email protected] with the clinical history, your diagnosis and diagnostic microscopic images in JPG, GIF or TIFF format (send as attachments, we will shrink if necessary). Please include any other images (gross, immunostains, etc.) that may be helpful or interesting. We will write the discussion (unless you want to), list you as the contributor, and send you $35 (US dollars) by check or PayPal for your time after we send out the case. Please only send cases with high quality images and a diagnosis that is somewhat unusual (or a case with unusual features).
Case of the Week #127
A 56 year old woman had a long history of untreated celiac sprue. An endoscopy showed nodular, ill defined areas in the antrum and gastritis. Small bowel and gastric biopsies were obtained.
stomach-other - modified Steiner stain
What is your diagnosis?
Stomach biopsies - MALT lymphoma, H. pylori gastritis
Small bowel biopsy - changes consistent with celiac disease
MALT lymphoma is characterized by a dense, monotonous population of centrocyte-like cells, lymphoepithelial lesions (infiltration of glandular epithelium by lymphocytes) and frequent follicular colonization. The tumor cells may also have plasmacytoid differentiation. Tumor cells are immunoreactive for B cell markers, including CD19, CD20, and CD79a, as well as bcl-10, with variable CD43 staining. They exhibit monoclonal light chain staining. This case demonstrated monoclonal heavy chain rearrangement by molecular studies.
Gastric MALT lymphomas are closely linked to Helicobacter pylori infection, and H. pylori eradication therapy produces a long term favorable outcome (Tohoku J Exp Med 2008;214:79)
Celiac disease, or gluten-sensitive enteropathy, is a T-cell mediated disease of genetically susceptible individuals, induced by ingesting proteins in wheat (gliadins), barley (hordeins) or rye (secalinin). Symptoms include episodic diarrhea, abdominal pain and distention and weight loss (Clin Med Res 2004;2:71), with clinical and microscopic improvement after dietary withdrawal. Microscopic changes include an increase in intraepithelial lymphocytes of 40+ lymphocytes/100 surface or upper crypt enterocytes, or early clustering of 12+ lymphocytes at the tip of villi and extending evenly down the sides of the villus (Mod Path 2003;16:342). There is also diffuse enteritis with marked atrophy or total loss of villi and elongated crypts. Definitive diagnosis requires these histologic findings plus positive serology and favorable clinical and serologic responses after dietary change. The differential diagnosis includes duodenal intraepithelial lymphocytosis with normal villous architecture, associated with H. pylori infection, but without any other features of celiac disease (Mod Path 2005;18:1134).
In this case, the presence of celiac sprue appears to be incidental. Celiac sprue is associated with an increased risk of malignant intestinal disease, but this is usually T cell intestinal lymphoma of the small bowel, not MALT lymphoma of the stomach. Celiac sprue does not appear to be associated with H. pylori gastritis (Am J Gastroenterol 2006;101:1880).
Nat Pernick, M.D., President
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