Whipple's disease

15 necropsied cases ofWhipple's disease are presented and compared with the literature. Most clinical manifestations of the disease are the result of three basic tissue alterations: a) Deposition of a strongly PAS-positive substance in macrophages of the small intestines, regional lymph nodes, and, to a lesser degree, extramesenteric visceral and peripheral lymph nodes, valvular endocardium, spleen, and other organs. Histochemical studies of the deposited material suggest a complex mucopolysaccharide with a protein and lipid component. b) Accumulation of a lymph-like lipid-containing substance within lymphatics and in extralymphatic cystic spaces of the intestinal mucosa and regional lymph nodes. c) Fibrosis in the regional lymph nodes, spleen, and mesenteric lymphatics as late phenomenon, probably incited by the deposited material. The degree of impairment of lymph drainage is clinically reflected in the severity of abdominal symptoms. Early diagnosis is best accomplished by transoral small bowel biopsy or by the examination of peripheral lymph nodes.