Tumoural form of Whipple's disease simulating carcinomatosis

Abstract

Whipple's disease is a rare disease caused by the actinomycete bacteria Tropheryma whipplei, which cause intestinal infection. The most common symptoms are chronic diarrhoea, weight loss, abdominal pain, arthritis and neurological abnormalities, which can be fatal. This paper reports a case of a 57-year-old Brazilian woman with diarrhoea, vomiting, abdominal pain, appetite loss, intermittent fever, malaise, weight loss and malnutrition. Migratory polyarthralgia and recurrent visual scotomas preceded the symptoms. The retroperitoneal pseudotumour formation finding was associated with prolonged wasting syndrome, which did not respond to usual therapies, thus leading to the investigation of carcinomatosis disease. After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. The patient improved clinically and started gaining weight after using ceftriaxone (IV).

Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections

“…Especially in the early phase of the disease, several less common symptoms of Whipple's disease can become manifest (Table 3) (120,(134)(135)(136)(137)(138). Dermatologists should be aware that both cutaneous (10, 139) and subcutaneous (140-143) lesions may be a manifestation of Whipple's disease, as cutaneous biopsy specimens containing PAS-positive macrophages characteristic of Whipple's disease have been reported (136).…”

“…If lymphadenopathy, anemia, and pancytopenia are present, apart from lymphoma of other nonspecific granulomatous reticuloendothelial disorders, Whipple's disease has to be considered as well (120,134,144). Retroperitoneal pseudotumor formation was found in a patient with loss of appetite, weight loss, malnutrition, malaise, abdominal pain, diarrhea, vomiting, and intermittent fever (135). After negative diagnostic results for malignancy, Whipple's disease was confirmed as a diagnosis, and the patient was treated with ceftriaxone, resulting in clinical improvement and weight gain (135).…”

“…Retroperitoneal pseudotumor formation was found in a patient with loss of appetite, weight loss, malnutrition, malaise, abdominal pain, diarrhea, vomiting, and intermittent fever (135). After negative diagnostic results for malignancy, Whipple's disease was confirmed as a diagnosis, and the patient was treated with ceftriaxone, resulting in clinical improvement and weight gain (135). There is an association between thrombocytopenia and T. whipplei infection, which probably results from peripheral platelet sequestration.…”