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与良性单克隆丙种球蛋白病(λ轻链)相关的淀粉样变性神经病

[Amyloid neuropathy associated with a benign monoclonal gammopathy (lambda light chains)].

作者信息

Graveleau P, Dairou R, Tritschler J C, Hénin D, Masson M, Cambier J

出版信息

Rev Neurol (Paris). 1978 Dec;134(12):773-80.

PMID:224439
Abstract

The authors report a case of non-familial amyloid neuropathy in which there was sensory-motor neuropathy of the 4 limbs, multiple lesions in the cranial nerves, and large subcutaneous amyloid deposits. Biopsy samples showed the presence of amyloid deposits in the interstitial tissues, the vessel walls in the muscle hypodermis, and in the bone marrow. A light monoclonal lambda chain was present in the serum. Immunofluorescent studies of the biopsy specimens showed the presence of elective fluorescence with an anti-lambda immunoserum in the amyloid substance.

摘要

作者报告了一例非家族性淀粉样变性神经病病例,该病例存在四肢感觉运动神经病、多条颅神经病变以及大量皮下淀粉样沉积物。活检样本显示间质组织、肌肉皮下血管壁和骨髓中存在淀粉样沉积物。血清中存在轻链单克隆λ链。活检标本的免疫荧光研究显示,淀粉样物质中存在抗λ免疫血清的选择性荧光。

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