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局限性胸腺淀粉样变性伴发重症肌无力:病例报告。

Localized thymic amyloidosis presenting with myasthenia gravis: case report.

机构信息

Department of Pathology, Chungbuk National University College of Medicine, Cheongju, Korea.

Department of Thoracic and Cardiovascular Surgery, Chungbuk National University College of Medicine, Cheongju, Korea.

出版信息

J Korean Med Sci. 2014 Jan;29(1):145-8. doi: 10.3346/jkms.2014.29.1.145. Epub 2013 Dec 26.

Abstract

A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.

摘要

一位 46 岁的女性患有重症肌无力(MG)2 个月,胸部 X 线偶然发现纵隔肿块。计算机断层扫描显示一个 4 厘米大小、边界清楚、分叶状的肿块,有结节状钙化,位于胸腺。显微镜下,肿块由弥漫性无定形嗜酸性物质组成。刚果红染色后,这些沉积物在偏振光显微镜下呈现出苹果绿双折射。免疫组化分析显示,κ 和 λ 轻链均为阳性,淀粉样 A 阴性。最终诊断为局限性原发性胸腺淀粉样变性。胸腺切除术后,MG 的症状得到控制,皮质类固醇用量减少。迄今为止,尚未有报道称 MG 与局限性胸腺淀粉样变性相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1987/3890467/b71859938e96/jkms-29-145-g001.jpg

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