Obliterative Lymphangitis of the Mesentery In Whipple's Disease

Abstract

Whipple's disease is a rare entity; to date, with the present case included, only some 70 cases have been reported in the literature. 1-56 A few of these cases, formerly considered to be examples of other conditions such as atypical sprue, chyle cysts of the mesentery, and mesenteric chyladenectasis, were in­ corporated into later reviews as representative examples of Whipple's disease. 9 Still others, first reported as Whipple's disease, have been discarded in subse­ quent reviews as exemplifying other entities or as incompletely substan­ tiated. 2 . 16 The vast majority of cases now accepted generally as authentic examples of Whipple's disease have been isolated instances, only seven groups of authors having had an opportunity to study more than one case.1, 16, 21, 26, 29, 31,54 Usu­ ally the disease is first recognized upon microscopic examination of autopsy material, so that detailed examination of pertinent features at gross autopsy, employment of certain special stains and chemical analyses, and the evaluation of therapeutic response are precluded. Such omissions, coupled with the rarity of the condition, have led to a divergence of opinion not only as to anatomic criteria for the diagnosis of Whipple's disease, but also as to pathogenesis. Most authors now agree that the pathologic anatomy is characterized by two principal lesions: (1) the mesenteric lymph nodes are involved in a cystic and lipogranuloma to us process; and (2) the mucosa of the small bowel is thick­ ened and infiltrated with large foamy macrophages. In addition, there mayor may not be overt evidence of chylous obstruction, i.e., dilated lacteals, dilated intestinal and mesenteric lymphatics and chylous ascites. Theories of pathogenesis, as tabulated by RUSSO,16 include the chemical or metabolic theory, which suggests defective fat metabolism or absorption, and the mechanical or inflammatory theory, which proposes an obstructive process involving some portion of the efferent lymphatic system. One of the stronger advocates of the mechanical or obstructive theory has been Clemmesen. 9 He postulated that the basic pathologic anatomy of Whip­ ple's disease is a chronic proliferative endolymphangitis involving predomi­ nantly the large efferent channels at the root of the mesentery. In the case pre­ sented below, this lesion initially proposed by Clemmesen is described for the