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Pathology of Whipple's Disease

"Macrophages containing diastase resistant PAS positive granules in the lamina propria of the small intestinal biopsy is key to the diagnosis"

Dr Sampurna Roy MD

 

 

      

Path Quiz Case 88: Case history and images

Diagnosis:  Whipple's Disease

 

                                                                                                                      

 

Whipple's disease is a rare chronic, relapsing, and multisystem disease caused by bacterial infection.

It was first described by George H. Whipple in 1907 and in 1949, the typical histological picture of the intestinal mucosa was described displaying foamy macrophages with cytoplasmatic periodic acid-Schiff (PAS) reactivity.

The etiology was further clarified in 1961 by detecting bacteria by electron microscopy.

Causative organism: Gram-positive organism named Tropheryma whipplei. The name of this bacterium—Tropheryma whipplei—is derived from Greek "trophe" (nourishment and food) and "eryma" (fence and barrier).

Age: Patients in fourth and fifth decade of life.

Clinical presentation:  May present with a variety of rather nonspecific symptoms like malabsorption, diarrhoea, weight loss and fever. The patient may also present with lymphadenopathy, hyperpigmentation and polyarthritis.

Sites affected: Intestine, central nervous system, joints, lymphnodes, lungs and heart.

Gross features:

The affected bowel is usually edematous with yellow plaque-like lesions and villiferous mucosa.

Affected lymph nodes appear yellow with a spongy cut surface.

Other affected organs, such as lungs and heart, may show plaques and edema.


Microscopic features: 

Small intestinal mucosa filled with distended macrophages in the lamina propria.

The macrophages contain diastase resistant PAS positive granules.

Villi may be distorted. 

Inflammation is usually absent.

Mesenteric lymphnodes may be involved and may show lymphatic dilatation indicating lymphatic obstruction.

Microscopic findings in the CNS include PAS-positive intracellular and extracellular organisms surrounded by reactive astrocytes.

The organisms may also violate the subarachnoid spaces and lead to the death of neurons, vacuolization, and demyelination.

Diagnosis: The diagnosis is usually established by small intestinal biopsy, which shows the pathognomonic periodic acid Schiff-positive infiltrates in the lamina propria.

PAS stain

The rod shaped bacilli may be demonstrated by electron microscopy.  

Polymerase chain reaction is now available to aid in the diagnosis of WD, and this organism has been identified by polymerase chain reaction in many body fluids, including cerebrospinal fluid, aqueous humor, and synovial fluid.

Detection in cerebrospinal fluid and peripheral blood is less consistent than in tissue. 

Differential diagnosis: Includes AIDS enteropathy caused  by Mycobacterium avium intracellulare (Ziehl-Neelsen staining positive).

Patients respond well to antibiotic therapy, but relapses occur.

 

Further reading

Bureš J, Kopáčová M, Douda T, et al. Whipple’s Disease: Our Own Experience and Review of the Literature. Gastroenterology Research and Practice. 2013;2013:478349.

Images of Whipple's Disease Website by Yale Rosen, MD. 

Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements.

Tropheryma whipplei infection. Colonization, self-limiting infection and Whipple's disease].

Whipple's disease: multiple systemic and neurological relapses.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 


 

 

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