Obliterative Lymphangitis of the Mesentery In Whipple's Disease

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