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肝活检中同时出现AL型淀粉样变性和轻链沉积病:一例报告

Simultaneous AL-type amyloid and light chain deposit disease in a liver biopsy: a case report.

作者信息

Smith N M, Malcolm A J

出版信息

Histopathology. 1986 Oct;10(10):1057-64. doi: 10.1111/j.1365-2559.1986.tb02542.x.

Abstract

A 57-year-old male caucasian presented with a peripheral neuropathy which had an autonomic component. Clinical examination revealed hepatomegaly and laboratory tests showed derangement of liver function tests and IgG lambda myeloma. Biopsy of the liver was performed. Histological examination revealed AL-type amyloid in the hepatic arteries and a perisinusoidal deposit of diastase resistant, periodic acid-Schiff positive material which did not react in the same way as the arterial deposit, giving no apple green birefringence when stained with Congo red. Immunohistochemistry showed the material to consist of lambda light chains. Electron microscopy confirmed that the material did not have the ultrastructural characteristics of amyloid. A diagnosis of light chain deposit disease concurrent with vascular AL-type amyloid was made.

摘要

一名57岁的白种男性因伴有自主神经成分的周围神经病变前来就诊。临床检查发现肝脏肿大,实验室检查显示肝功能检查结果异常以及存在IgG λ型骨髓瘤。进行了肝脏活检。组织学检查显示肝动脉中有AL型淀粉样蛋白,以及窦周有抗淀粉酶、过碘酸希夫染色阳性的物质沉积,该物质的反应方式与动脉沉积物不同,刚果红染色时无苹果绿双折射。免疫组织化学显示该物质由λ轻链组成。电子显微镜检查证实该物质不具有淀粉样蛋白的超微结构特征。诊断为并发血管AL型淀粉样蛋白的轻链沉积病。

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