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Whipple’s Disease: Report of Case Apparently Cured and Discussion of the Histochemical Features

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Abstract

INTESTINAL lipodystrophy, first described by Whipple,1 is a rare disorder, the clinical manifestations of which are less well defined than are the pathologic features. Review articles2,3,4,5 indicate that the onset of the disease is insidious, marked by periodic polyarthritis or arthralgia and, at times, chills and fever. Bowel symptoms vary and, although diarrhea usually appears at some time during the course of the disease, it often is not a prominent complaint or may not occur until late; frequently, constipation may be present. Loss of weight, weakness, fatigability, and general debility are common manifestations. Physical findings may include pigmentation of exposed areas of skin, hypotension, evidence of loss of weight and, frequently, peripheral lymphadenopathy. A palpable, abdominal mass may be present, and there may be a small amount of ascitic fluid. Laboratory examination of the blood frequently reveals a hypochromic or a microcytic anemia and a normal leukocyte count. The roentgeno-graphic findings are not pathognomonic, although disturbances of intestinal motility and alteration of the usual mucosal pattern of the small intestine are not uncommon.

Because of these clinical and laboratory manifestations, differentiation from the sprue syndrome, Addison’s disease, or rheumatoid arthritis is often difficult; in fact, few examples of a correct antemortem diagnosis of Whipple’s disease are recorded.

Histologically, the lesions of the disease are characteristic. They consist of a marked infiltration of the lamina propria of the small intestine by histiocytes that frequently line cystic spaces of varying size. Although these latter are sudanophilic, the histiocytes fail to stain with . . .


 

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