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Whipple’s Disease
It is a systemic infectious disease caused by an actinomycete, Tropheryma whippelii affecting the triad of small intestine, CNS and joints.
The bacteria characteristically proliferate inside the macrophages without getting destroyed.
Whipple disease interferes with normal digestion by impairing the breakdown of foods, and hampering your body's ability to absorb nutrients, such as fats and carbohydrates.
Features
- The hallmark feature of the disease is small intestinal mucosa having macrophages in the lamina propria and these macrophages show the presence of PAS positive, diastase resistant granules and rod shaped bacteria on electron microscopy.
- There is mucosal edema, dilation of the lymphatics and involvement of mesenteric lymph nodes.
- The macrophages having the bacteria can also be found in the joints, brain, cardiac valves etc with absence of inflammation being a typical feature.
- Clinical features include arthropathy (initial presentation), diarrhea, weight loss, hyperpigmentation and dementia. The diarrhea is due to impaired lymphatic transport.
- Diagnosis is confirmed by identification of T. Whipplei by polymerase chain reaction (PCR).
- Presence of PAS positive macrophages containing the characteristic small bacilli. But, similar picture (PAS +ve macrophages with bacilli) can also be seen with M. Avium complex (cause of diarrhea in AIDS).
- These organisms are acid fast whereas Tropheryma is not.
- The organs in which these foamy macrophages can be seen are Liver, Small intestine, Lymph nodes, Heart, Eyes, CNS and synovial membranes of joints.
High magnification micrograph showing the characteristic foamy macrophages in the lamina propria, H&E stain
