Small bowel (small intestine)
Infectious disorders
Whipple disease


Topic Completed: 1 August 2012

Revised: 12 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Whipple disease[TIAB] small bowel

Hanni Gulwani, M.B.B.S.
Page views in 2018: 7,873
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Cite this page: Gulwani H. Whipple disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/smallbowelwhipple.html. Accessed July 17th, 2019.
Definition / general
  • 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
Diagrams / tables

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Pathophysiology of Whipple disease

Diagnosis
  • PCR, immunohistochemistry, PAS stain (PAS+ diastase resistant)
Case reports
Treatment
  • Antibiotics
  • Relapses are common
Gross description
  • Shaggy mucosa, edematous bowel wall
Microscopic (histologic) description
  • Distended macrophages in lamina propria containing PAS+ (diastase resistant) 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 stain, resembling Gaucher cells
Microscopic (histologic) images

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Contributed by Drs. Derek Mathis and Rosemarie Rodriguez


Various images


PASD images



Case of the Week #303 (case #1)

H&E


PAS stain



Case of the Week #303 (case #2)


H&E


PAS stain

Positive stains
Electron microscopy description
  • Rod shaped bacteria
Electron microscopy images

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52 year old man

Differential diagnosis
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