Suppr超能文献

[一例经肌肉活检确诊的系统性AL淀粉样变性病例]

[A case of systemic AL amyloidosis diagnosed on muscle biopsy].

作者信息

Yata Tomohiro, Miwa Takashi, Araki Katsuya, Kida Toru, Toyooka Keiko, Nishino Ichizo, Tatsumi Chikao

机构信息

Department of Neurology, Toyonaka Municipal Hospital.

Department of Neurology, Osaka Toneyama Medical Center.

出版信息

Rinsho Shinkeigaku. 2020 Jan 30;60(1):60-63. doi: 10.5692/clinicalneurol.cn-001339. Epub 2019 Dec 17.

Abstract

A 69-year-old man was admitted to our hospital with a 1-year history of progressive easy fatigability while walking. He presented with proximal muscle weakness dominant in the lower extremities, hoarseness, and mild dysphagia. Muscle pseudo-hypertrophy was observed in the gastrocnemius. A biopsy specimen from the left deltoid muscle revealed amyloid deposition in the blood vessels and ring-like fibers. These findings suggested amyloid myopathy. The serum and urine immunofixation electrophoresis detected κ type Bence-Jones proteins, and bone marrow examination showed an increase in atypical plasma cells; thus, we established a diagnosis of multiple myeloma. Thereafter, he experienced frequent diarrhea, and the gastrointestinal endoscopy revealed extensive amyloid deposition in the upper and lower digestive tract. We started treatment with lenalidomide and dexamethasone; however, his condition worsened, and he died of aspiration pneumonia. Amyloid myopathy indicated systemic AL amyloidosis; therefore, muscle biopsy was necessary in this case.

摘要

一名69岁男性因行走时渐进性易疲劳1年入住我院。他表现为以双下肢为主的近端肌无力、声音嘶哑和轻度吞咽困难。腓肠肌可见肌肉假性肥大。左三角肌活检标本显示血管内淀粉样沉积和环状纤维。这些发现提示为淀粉样肌病。血清和尿免疫固定电泳检测到κ型本-周蛋白,骨髓检查显示非典型浆细胞增多;因此,我们确诊为多发性骨髓瘤。此后,他频繁腹泻,胃肠内镜检查显示上、下消化道广泛淀粉样沉积。我们开始使用来那度胺和地塞米松治疗;然而,他的病情恶化,最终死于吸入性肺炎。淀粉样肌病提示全身性AL淀粉样变性;因此,本例有必要进行肌肉活检。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验