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系统性淀粉样变性伴肠系膜肿块,该肿块表现为蛋白性淋巴结病。

Systemic amyloidosis with a mesenteric mass which had the appearance of proteinaceous lymphadenopathy.

作者信息

Oyaizu N, Shikata N, Tsubura A, Morii S, Taniguchi N, Miura K

出版信息

Acta Pathol Jpn. 1987 Jan;37(1):133-9. doi: 10.1111/j.1440-1827.1987.tb03141.x.

Abstract

An autopsy case of systemic amyloidosis in a 46-year-old Japanese female who had M component in sera but no manifestations of myeloma is described. Autopsy revealed massive milky ascites and a large mesenteric mass, which showed histologically perivascular concentric deposition of eosinophilic materials with mixture of residual follicles and scattered plasma cells. These morphological findings closely resembled those seen in proteinaceous lymphadenopathy, and the material in the mesenteric mass proved to be an amyloid A lambda protein. Subsequent immunohistochemical observations strongly suggested that this mass lesion was a "burnt-out" plasmacytic dyscrasia.

摘要

描述了一名46岁日本女性系统性淀粉样变性的尸检病例,该患者血清中有M成分但无骨髓瘤表现。尸检发现大量乳糜性腹水和一个大的肠系膜肿块,组织学显示嗜酸性物质呈血管周围同心性沉积,伴有残留滤泡和散在浆细胞混合存在。这些形态学发现与蛋白质性淋巴结病所见非常相似,肠系膜肿块中的物质被证实为淀粉样Aλ蛋白。随后的免疫组化观察强烈提示该肿块病变是一种“消退型”浆细胞发育异常。

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引用本文的文献

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Diffuse mesenteric amyloidosis.弥漫性肠系膜淀粉样变性
Dig Dis Sci. 1997 May;42(5):1079-82. doi: 10.1023/a:1018809724577.

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