Abstract
The etiology and pathogenesis of Whipple's Disease (lipodystrophy intestinalis), a clinical syndrome similar to sprue, has been a subject of speculation since its original description. 1 As its synonym implies, it is believed by most observers to represent an obscure disturbance of fat metabolism.
Black-Schaffer, Hendrix and Handler 2 reported a study of 4 cases which led them to the following conclusion: The disease, in contrast to sprue, may be readily recognized, anatomically, by non-lipid macrophagocyto-sis in the lamina propria of the small intestine and occasionally the proximal colon, lipogranulomatosis of the mesenteric lymph nodes, absence of significant evidence of chylous obstruction; and clinically, poor fat, glucose and probably protein absorption and the absence of macrocytic anemia.
The characteristic intestinal lesion is a crowding of the lamina propria by macrophages containing an isotopic refractile substance which Whipple found unstainable with osmic acid. This observation has been repeatedly overlooked in the literature, almost all authors assuming a lipid nature for this curious substance. The study of Black-Schaffer, Hendrix and Handler confirmed Whipple's observation. In 3 cases∗ the phagocytosed material did not stain with Sudan IV, and 2† were likewise negative with Nile blue sulphate as well as osmic acid. Chemical analysis of the intestinal mucosa of two cases revealed no increase, over normal controls, of the lipid content.
The characteristic enlarged, cystic, fat-filled mesenteric lymph nodes (lipogranulomatosis) are so prominent that they have dominated the approach to the problem.
Careful examination of the nodes revealed, 2 adjacent to sudanophilic macrophages, others containing sudanophobic substance similar in all respects to that described in the intestine. As a consequence of the pathologic anatomy, the histochemistry and the chemical analyses, a pathogenesis of the lesions was proposed. 2
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