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12 March 2014 - Case of the Week #303

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Thanks to Dr. Raul Gonzalez, Vanderbilt University Medical Center, and Dr. Don Xu, University of California, San Diego, for contributing these cases. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.

This intensive review is designed for practicing pathologists and other interested physicians who have the desire to remain abreast of current issues in surgical pathology. The purpose of this unique course is to provide participants with overviews of selected topics of continuing interest and updates on methods for solving diagnostic problems encountered in surgical pathology. The program will consist of a series of 50 minute lectures by nationally recognized pathologists, and the format will include clinical-pathologic correlations and question and answer sessions.

CME and SAMs Credit Available.


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Case of the Week #303

Case #1 (contributed by Dr. Raul Gonzalez)

A 50 year old man presented with upper GI bleeding and friable duodenal mucosa on endoscopy.


PAS stain

Case #2 (contributed by Dr. Don Xu)
A 47 year old man with a history of chronic arthralgias for two years presented with fevers, abdominal pain, and weight loss. He was found to have microcytic anemia and mesenteric and retroperitoneal lymphadenopathy. The pictures of duodenal biopsy are shown.


PAS stain

What is your diagnosis?

Whipple's disease

Both cases showed blunted villi and expansion of the lamina propria by foamy histiocytes. Periodic acid Schiff (PAS) stain revealed numerous microorganisms within macrophages. In case #1, unstained slides were sent to an outside institution for Tropheryma whipplei immunostain, which was positive. In case #2, PCR study confirmed Tropheryma whipplei infection.

Whipple's disease is a rare systemic infection due to Tropheryma whippelii, a gram positive intracellular actinomycete (World J Gastroenterol 2009;15:2078), first described in 1907 by Dr. George Hoyt Whipple. Dr. Whipple won the Nobel Prize (jointly) in 1934 for another discovery, that liver given as food to dogs reversed the anemia, leading to treatment of pernicious anemia. He was friends of Dr. Allen Whipple, a surgeon who described the Whipple procedure and the Whipple's triad of insulinoma.

Whipple's disease, also called intestinal lipodystrophy, typically affects white men ages 30-49 years, in the proximal intestine and mesenteric lymph nodes. Patients typically present with malabsorption (diarrhea, weight loss, abdominal pain), and occasionally polyarthritis, lymphadenopathy, hyperpigmentation and CNS complaints. Histology, in addition to the above features, shows dilated lymphatics or fat vacuoles. It may show multinucleated giant cells. EM shows rod-like organisms.

The differential diagnosis includes:
Histoplasmosis: faint blue dot-like inclusions surrounded by a clear halo, PAS or silver stain shows budding yeast
Mycobacterium avium-intracellulare infection: immunocompromised patients, patchy infection, no lipid vacuoles, PAS shows faintly positive bacillary forms
• Mineral oil ingestion

Treatment is with antibiotics, which must be continued for at least a year, or there is a high rate of relapse.

Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
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