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伴有周围神经病变的华氏巨球蛋白血症——IgM M蛋白在自身免疫机制中的作用

Waldenstróm's macroglobulinemia with peripheral neuropathy--the role of IgM M-protein in autoimmune mechanism.

作者信息

Wang C R, Chiu H C, Hsieh H C, Hsieh R P, Wu Y C, Chuang C Y

机构信息

Department of Internal Medicine, National Taiwan University Hospital, Taipei, ROC.

出版信息

Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi. 1988 Feb;21(1):9-15.

PMID:2850141
Abstract

A total of 11 cases of Waldenstróm's Macroglobulinemia (WM) have been investigated to study the association of the disease with neuropathy. A 53-year-old female patient (Wu) had a pronounced peripheral neuropathy, in which the role of IgM M-protein could be observed. Sections of Wu's biopsied sural nerve were subjected to light and electron microscopy and direct immunofluorescence (IF). Myelin degeneration with axonal change was found, while fluorescence reaction occurred in epineurium only. Sections of a healthy dog's sciatic nerve were submitted to purified IgM M-proteins from Wu and another WM patient (Kon) without neuropathy, respectively. Indirect IF showed a deposit of fluorescence on the myelin sheath by Wu's, but not by Kon's, M-proteins. It is proposed that an autoimmune mechanism underlies the peripheral neuropathy in the WM patient.

摘要

共对11例华氏巨球蛋白血症(WM)患者进行了研究,以探讨该疾病与神经病变之间的关联。一名53岁女性患者(吴)患有明显的周围神经病变,其中可观察到IgM M蛋白的作用。对吴的腓肠神经活检切片进行了光镜、电镜及直接免疫荧光(IF)检查。发现有髓鞘变性伴轴突改变,而仅在神经外膜出现荧光反应。分别将来自吴和另一名无神经病变的WM患者(孔)的纯化IgM M蛋白作用于健康犬的坐骨神经切片。间接免疫荧光显示,吴的M蛋白可使髓鞘出现荧光沉积,而孔的M蛋白则无此现象。提示WM患者周围神经病变存在自身免疫机制。

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