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Small bowel (small intestine)

Infectious disorders

Whipple’s disease


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 13 March 2014, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare systemic infection due to Tropheryma whippelii, a gram positive intracellular actinomycete (World J Gastroenterol 2009;15:2078)
● Usually affects proximal intestine, mesenteric lymph nodes
● Also called intestinal lipodystrophy
● Usually white males ages 30-49 years

Symptoms:
● Malabsorption with diarrhea, weight loss, abdominal pain
● Occasionally polyarthritis, CNS complaints, lymphadenopathy, hyperpigmentation
● Cardiac involvement less common
● Fatal without antibiotics

Pathophysiology
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Pathophysiology of Whipple's disease

Diagnosis
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● PCR, immunohistochemistry, PAS stain

Treatment
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● Antibiotics
● Relapses are common

Case reports
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● 47 and 50 year old men with malabsorption (Case of the Week #303)

Gross description
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● Shaggy mucosa, edematous bowel wall

Micro description
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● Distended macrophages in lamina propria containing PAS+ granules and rod shaped bacilli by EM
● Dilated lymphatics or fat vacuoles
● Often multinucleated giant cells; rarely epithelioid granulomas in minority
● In mesentery or retroperitoneal nodes, resembles lipogranulomatous inflammation with round empty spaces

Note:
● After treatment, macrophages decrease in lamina propria but remain present elsewhere for years; cytoplasmic inclusions become tissue-paper like with PAS, resembling Gaucher’s cells

Micro images
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Various images (contributed by Drs. Derek Mathis and Rosemarie Rodriguez, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas)


PASD images (contributed by Drs. Derek Mathis and Rosemarie Rodriguez, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas)

Case of the Week #303 (Case #1):

H&E


PAS stain


Case of the Week #303 (Case #2):



H&E


PAS stain

Positive stains
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● PAS, anti-T Whippelii immunostain (Hum Pathol 2003;34:589)

Electron microscopy description
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● Rod-shaped bacteria

Electron microscopy images
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52 year old man

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

End of Small bowel (small intestine) > Infectious disorders > Whipple’s disease


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