Lymphedema, lymphocytic myocarditis, and sarcoidlike granulomatosis: manifestations of Whipple's disease

Article Abstract:

Whipple's disease is a rare infectious disease that can be positively diagnosed by microscopic examination of abdominal tissue. A characteristic staining pattern is found with a particular cell stain called periodic acid Sciff reagent, and bacteria are detectable within the tissues. Most of these patients demmonstrate poor intestinal absorption, as the small intestine is generally affected, resulting in a fatty stool, abdominal pain, weight loss, fever, arthritis and inflammation of the spleen and lymph nodes. Often the Whipple's disease patient has a progressive collection of tissue fluid and a large number of lymphocytes, a type of white blood cell, inside the muscular walls of the heart. This report details the clinical signs, symptoms and ultimate autopsy findings of a 44-year-old man. The patient showed abnormal collection of tissue fluid in his hands, feet and ankles. There was also evidence that the lymph nodes of his groin were involved. A biopsy of a node showed a sarcoid-like granuloma, but his bowel biopsy appeared normal. Biopsy of the skin, bone marrow and liver were all normal. The patient was treated with the anti-inflammatory agent prednisone, but his fever returned. Ultimately he was placed on the analgesic compound ibuprofin as an anti-inflammatory therapy. For three years, the patient had no symptoms except for the excess tissue in his extremities, which gradually extended to his chest, face and eye sockets. Then the other symptoms returned. Chest X-rays and CAT scans showed changes to the lungs, lung membranes and the collection of fluid around the lungs. Ultrasound examination of his heart showed thickening which was indicative of myocarditis or inflammation of the heart muscle. He was treated with prednisone to reduce the inflammatory condition and was discharged. He improved for five months, but then developed heart difficulties that required a pace-maker. After a period of steady improvement, he suddenly died. At autopsy, the finding of Whipple's disease was confirmed. The unusual aspects of this case should be noted by physicians.

Case studies, Causes of, Myocarditis, Lymphedema

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Tropheryma whippelii DNA is rare in the intestines of patients without other signs of Whipple disease

Article Abstract:

The bacterium that is believed to cause Whipple's disease was not found in intestinal biopsy samples of 342 people who had no symptoms of Whipple's disease. This strengthens the belief that the bacterium is responsible for causing Whipple's disease, a gastrointestinal disease that also affects joints, lymph nodes, and the nervous system.

Research

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Culture and Immunological Detection of Tropheryma whippelii From the Duodenum of a Patient With Whipple Disease

Article Abstract:

A technique is described for detecting Tropheryma whippelli in intestinal tissue samples using a mouse antibody against the organism. This bacterium causes Whipple disease. The test detected the organism in a patient with Whipple disease but not in intestinal tissue samples from 10 healthy people.

Diagnosis

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