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[λ轻链骨髓瘤中的假性肥厚性骨盆-小腿淀粉样变肌病。临床、形态学及免疫细胞化学研究]

[Pseudo-hypertrophic pelvi-crural amyloid myopathy in lambda light-chain myeloma. Clinical, morphological and immunocytochemical study].

作者信息

Pellissier J F, Aquilina C, Murisasco A, Toga M

出版信息

Rev Neurol (Paris). 1987;143(2):128-36.

PMID:3110902
Abstract

The case of 65 year old woman with progressive enlargement and "wooden" induration of the pelvic girdle and thigh muscles due to an amyloid infiltration is reported. Muscle changes appeared two years after a diagnosis of myeloma with free lambda light chains. The patient complained of muscle pain, lassitude and weakness. Macroglossia was present. Skeletal muscle (vastus lateralis) contained large amounts of amyloid substance and showed type 2B atrophy. There was no fiber type grouping. Some amyloid deposits abutted on the muscle fiber, destroyed the basal lamina and sarcolemma, but never infiltrated it. Besides the amyloid phagocytosis by macrophages, a relationship between amyloid filaments and fibroblasts was present. Another non-congophilic substance was revealed using the Avidin-Biotin peroxidase complex to localize lambda light chains by light microscopy and corresponded to a granular substance in electron microscopy. Clinicopathological results are discussed with a review of thirteen similar cases previously reported.

摘要

报告了一名65岁女性病例,因淀粉样蛋白浸润导致骨盆带和大腿肌肉进行性肿大及“木质样”硬结。肌肉改变出现在诊断为伴有游离λ轻链的骨髓瘤两年后。患者主诉肌肉疼痛、疲倦和虚弱。存在巨舌症。骨骼肌(股外侧肌)含有大量淀粉样物质,并显示2B型萎缩。无纤维类型群组化现象。一些淀粉样沉积物邻接肌纤维,破坏基膜和肌膜,但从未浸润其中。除了巨噬细胞对淀粉样蛋白的吞噬作用外,淀粉样细丝与成纤维细胞之间也存在关联。使用抗生物素蛋白-生物素过氧化物酶复合物通过光学显微镜定位λ轻链时,发现了另一种非嗜刚果红物质,在电子显微镜下对应于一种颗粒状物质。结合先前报道的13例类似病例对临床病理结果进行了讨论。

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