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经支气管镜检查诊断为肺淀粉样变性合并肺含铁血黄素沉着症。

Pulmonary amyloidosis complicated with pulmonary hemosiderosis, diagnosed with bronchoscopy.

作者信息

Ogo Naruhiko, Yanagihara Toyoshi, Nishimura Ryota, Mannoji Hiroshi, Yoneda Reiko, Hayashi Masayasu, Egashira Ayaka, Asoh Tatsuma, Maeyama Takashige

机构信息

Department of Respiratory Medicine, Hamanomachi Hospital, Fukuoka, 810-8539, Japan.

Department of Cardiovascular Medicine, Hamanomachi Hospital, Fukuoka, 810-8539, Japan.

出版信息

Respir Med Case Rep. 2021 Mar 19;33:101400. doi: 10.1016/j.rmcr.2021.101400. eCollection 2021.

Abstract

We describe a case of an 82-year-old Japanese woman with pulmonary amyloidosis and hemosiderosis associated with multiple myeloma. She had a background of end-stage renal failure of unknown etiology and had been on maintenance dialysis for 2 years. She complained of exertional dyspnea for four months. High-resolution CT of the chest revealed diffuse ground-glass opacities with mosaic attenuation, consolidation in the left lingular lobe, and wedge-shaped, subpleural nodules in the bilateral lower lobes. A transbronchial lung biopsy of the left lingular lobe showed deposition of amorphous, eosinophilic amyloid at the smooth muscle layer of bronchial tissue, with a positive Congo red staining signal in polarized light. Bronchoalveolar lavage fluid was brownish-yellow, and numerous hemosiderin-laden macrophages were detected with Berlin blue staining. From these findings, a diagnosis of pulmonary amyloidosis complicated with pulmonary hemosiderosis was made. Further work-up led to a diagnosis of multiple myeloma. Pulmonary amyloidosis complicated with pulmonary hemosiderosis is a rare disorder and may be underdiagnosed. Physical examination, such as the appearance of the tongue, can assist the diagnosis of systemic amyloidosis. Use of bronchoscopy allows physicians make an early diagnosis of pulmonary amyloidosis that is minimally invasive.

摘要

我们描述了一例82岁的日本女性患者,患有与多发性骨髓瘤相关的肺淀粉样变性和含铁血黄素沉着症。她有病因不明的终末期肾衰竭病史,已接受维持性透析2年。她主诉四个月来劳力性呼吸困难。胸部高分辨率CT显示弥漫性磨玻璃影伴马赛克样衰减、左舌叶实变以及双侧下叶楔形胸膜下结节。左舌叶经支气管肺活检显示支气管组织平滑肌层有无定形嗜酸性淀粉样物质沉积,偏振光下刚果红染色信号阳性。支气管肺泡灌洗液呈棕黄色,柏林蓝染色检测到大量含铁血黄素巨噬细胞。根据这些发现,诊断为肺淀粉样变性合并肺含铁血黄素沉着症。进一步检查确诊为多发性骨髓瘤。肺淀粉样变性合并肺含铁血黄素沉着症是一种罕见疾病,可能诊断不足。体格检查,如观察舌头外观,有助于系统性淀粉样变性的诊断。支气管镜检查的应用使医生能够以微创方式早期诊断肺淀粉样变性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/34e3/8024703/fc4aea78dc23/gr1.jpg

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