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Femme de 63 ans se plaignant d'une diarrhe depuis 2 mois, avec perte de poids (12Kg en 3 mois), et douleurs articulaires. Biopsie duodnale.

 

63 yo female complaining of a diarrhea for 2 months, weigth loss (12 Kg in 3 months), and joint pains.Duodenal biopsy performed.

 
     
  Faible grossissement, villosités intestinales trappues, légèrement de hauteur réduite.   Scanning view, enlarged intestinal villi, with a preserved general architecture.Villi are slightly reduced in height.  
     
  Chorion infiltré par des nappes de cellules larges, à cytoplasme pale.   Lamina propria is infiltrated by sheets of large cells.The cells have a pale, more or less eosinophilic cytoplasm  
     
  Epithélium intestinal conservé. Les cellules a cytoplasme vacuolaire présentent les caractères de macrophages.   The intestinal epithelium is preserved.The cells have an ovoid regular nucleii, a vacuolar cytoplasm, are macrophages.  
     
  Ils sont négatifs au Bleu Alcian, et positifs au PAS.   The macrophages are negative to Alcian Blue stain (Goblet cells +).PAS stain discloses diffuse positivity of the macrophages.  
     
  La positivité du cytoplasme est intense, granulo-vacolaire, diastase resistante.   Positivity of the cytoplasm is intense and granular or vacuolar.  
  Diagnostic Proposé:

 

 

 

 

 

 

 

 

 

Maladie de Whipple

  Proposed Diagnosis:

 

 

 

 

 

 

 

 

 

Whipple's disease

 
         

Whipple's Disease


CLINICAL FEATURES

This is a rare bacterial infection attributed to Tropheryma whippelii which affects the small bowel in a diffuse fashion but can also involve many other systems.There may be an underlying immunologic defect in these patients, possibly genetically determined, but this has not been clearly established.

Typical patients are middle-aged women, who present with fever, malabsorption and weight loss, arthritis, and Iymphadenopathy. Central nervous system manifestations, including ophthalmoplegia and personality change, have been reported in about 10% of patients.

 

ENDOSCOPIC FINDINGS

The mucosal folds appear thickened and are patchily coated with yellow-white material.

On closer inspection, these patches or plaques may represent enlarged bulbous villi. Previously, it was believed that the proximal small bowel was always affected in a diffuse fashion. However,some patients may have only spotty involvement of the lamina propria, and some biopsy specimen may not contain any characteristic macrophages. Direct viewing endoscopy offers an advantage because the focal yellow areas can be selectively biopsied.

 

Differential diagnosis

Histologically, the only other disorder that may cause a problem in differential diagnosis is Mycobacterium avium-intracellulare in patients with AIDS. Acid-fast stains should therefore be done when macrophages are present in the lamina propria. Massive infection with M. avium has been termed pseudo- Whipple's disease, but the macrophages look completely different, even at the light microscopic level.

In AIDS in the absence of M. avium, small clumps of unexplained PAS-positive macrophages are occasionally observed in the small bowel mucosa, but they are very patchily distributed and the intensity of their PAS positivity is variable.

Histoplasmosis may be associated with a macrophage infiltrate, but if that is part of the differential diagnosis clinically, then fungal stains should be done.

Involvement of the colon is extremely rare in Whipple's disease.

 

(Ref. Ackerman's Surgical Pathology, 8th Ed.)