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表现为黄白色多结节性支气管内突起、伴不规则血供、易出血的胸内淀粉样肿瘤。

Intrathoracic amyloid tumors that presented as yellowish multinodular endobronchial protrusions with irregular vascularity and easy bleeding.

机构信息

Department of Respiratory Medicine, Sasebo City General Hospital, Nagasaki, Japan.

Department of Pathology, Sasebo City General Hospital, Nagasaki, Japan.

出版信息

Thorac Cancer. 2019 Oct;10(10):2026-2030. doi: 10.1111/1759-7714.13159. Epub 2019 Aug 4.

Abstract

Immunoglobulin light-chain (AL) amyloidosis is a monoclonal plasma cell neoplasm that has a tendency to bleed easily. However, the potential risks of transbronchial biopsy in such cases have not been fully proven. Here, we report a case of parotid and intrathoracic AL amyloid tumors that presented as endobronchial protrusions that bled easily. Bronchoscopy under conventional white light and narrow band imaging revealed yellowish multinodular protrusions, in which irregular tortuous or dotted vessels were observed. Unexpectedly, biopsy of the lesion resulted in persistent bleeding. The biopsy specimen showed a large amount of amyloid deposition and calcification directly under the bronchial epithelium, as well as amyloid deposits in the blood vessel walls. In patients suspected to have amyloidosis, the presence of yellowish multinodular endobronchial protrusions, particularly with irregular vascularity, should prompt careful attention to avoid fatal postprocedural bleeding.

摘要

免疫球蛋白轻链(AL)淀粉样变性是一种单克隆浆细胞肿瘤,容易出血。然而,此类情况下经支气管活检的潜在风险尚未得到充分证实。在此,我们报告一例腮腺和胸内 AL 淀粉样肿瘤病例,表现为易出血的支气管内隆起。常规白光和窄带成像支气管镜检查显示出黄色多结节状隆起,其中可观察到不规则扭曲或点状血管。出乎意料的是,病变活检导致持续出血。活检标本显示大量的淀粉样物质直接沉积在支气管上皮下,以及血管壁中的淀粉样物质沉积。在疑似淀粉样变性的患者中,存在黄色多结节性支气管内隆起,特别是伴有不规则血管时,应引起高度警惕,以避免致命的术后出血。

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