结节性与气管支气管淀粉样变性合并的罕见病例。
A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis.
作者信息
Ding Feihong, Li Yun, Balasubramanian Shailesh, Ghosh Subha, Valent Jason N, Almeida Francisco, Arrossi A Valeria, Mehta Atul
机构信息
Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
出版信息
Oxf Med Case Reports. 2021 Feb 15;2021(2):omaa134. doi: 10.1093/omcr/omaa134. eCollection 2021 Feb.
Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.
淀粉样变性是一组异质性疾病，其特征是错误折叠的蛋白质在细胞外沉积，可全身性或局部性累及单一系统。肺淀粉样变性较为罕见，根据受累的解剖部位可分为三种形式：结节性肺淀粉样变性、气管支气管淀粉样变性和弥漫性肺泡间隔淀粉样变性。前两种通常代表局限性淀粉样疾病，后者代表全身性疾病。典型的肺实质和气管支气管淀粉样变性在局限性肺淀粉样变性中通常不会同时出现。在此，我们报告一例独特的局限性肺免疫球蛋白轻链淀粉样变性病例，表现为实质结节和气管支气管淀粉样沉积。
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