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小腸全域にリンパ装置が増殖する汎発性結節性リンパ濾胞増殖症は,無ガンマグロブリン血症を示すことで一応独立した疾患として認められる.また発症原因がいまだに明確ではないが,蛋白漏出性胃腸症の1つとして小腸のリンパ管拡張症も独立した症候群として認められているかと思われる.
われわれはこの2つの疾患が併存し,高度の低蛋白血症を来たした症例をすでに発表したが5),この同一症例に,その後の検討で小腸の非特異性多発性潰瘍も併存していることが判明したので追加報告したいと思う.
The patient is a 32 years old female with hypoproteinemia and anemia. Gastro-intestinal series showed numerous nodular filling defects in the ileum, ranging from 2 to 3 mm in diameter. A few unusual localized constrictions were found at the proximal ileum. They showed multiple unilateral and annular configuration. The mucosal relief converged toward the constrictions.
Gross observation of the resected specimens showed numerous nodular polypoid lesions, from 2 to 3 mm in diameter, and irregular folds on the surface of the mucosa of localized hypertrophic intestinal wall.
Histopathological examination of all the resected specimens revealed hyperplasia of solitary lymph follicles in the polypoid lesions. Dilated submucosal lymphatic channels at the tips of the villi and erosions of the mucosa were seen. Hyperplasia of the fibrous connective tissue at the localized hypertrophic wall was limited to the submucosal layer. Ulcers, active and/or covered with the regenerating epithelium, were observed on its surface.
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