The Whipple Riddle

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Abstract
Whipple disease (intestinal lipodystrophy) is a systemic disorder that involves not only the small bowel and lymph nodes but also the liver, spleen, heart, brain, and other organs. Histopathologically, the disease is characterized by the ubiquitous macrophages that give a positive diastase-resistant PAS staining reaction. Although Whipple disease can be treated with antibiotics, its etiology remains one of the unsolved riddles of medicine, that is, perhaps, until now. Clancy and associates1recently reported in theBritish Medical Journalthat a cell wall-deficient form (CWD) of an α-hemolyticStreptococcuswas grown from a prolonged monolayer cell culture of a lymph node taken from a patient with Whipple disease. Serological cross reactivity was shown between the organisms and the material within Whipple disease macrophages. In vitro studies characterized the organisms as a facultative intracellular parasite that caused the accumulation within cells of PASpositive material. Moreover, parenteral injection of this CWD organism into

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