2例气管支气管淀粉样变性分析

ANALYSIS OF 2 CASES TRACHEOBRONCHIAL AMYLOIDOSIS

目的探讨气管及支气管淀粉样变性的临床表现、病理学、影像学特征和诊疗方法。方法总结广州市第一人民医院的2例气管支气管淀粉样变性病例特点,并结合相关文献进行分析和总结。结果①患者主要临床表现为反复咳嗽、咳痰、咳血及气促。②影像学特点:CT提示双侧假声带、气管管壁明显肿胀狭窄及环形增厚。③病理学检查提示气管粘膜上皮镜下可见大量片、块状红染的均质"云雾状"无结构物质沉积,淀粉染色阳性。④治疗:气管淀粉样变性的治疗有以马法兰+泼尼松治疗为主的化疗、激光、冷冻及手术治疗或对症治疗。结论该病临床表现无明显特征,容易误诊,病理学是确诊的唯一手段,治疗困难,往往预后不良。

Objective This article aims at studying the clinical features, pathology,imageology features and treatment. Methods Features Of 2 cases diagnosed in The First Municipal Hospital of Guangzhou were summarized while referring to related literature review. Results ①The main clinical manifestations included cough, sputum, he- moptysis and short breath.②Imageology features, CT find irregular luminal narrowing and airway wall thickening of bilateral vocal cords and trachea. ③Pathology Inspection shows large amount of homogeneous patchy structureless substance which are stained positive under tunica mucosa epithelial cells. ④Treatment, The treatment of the disease included chemotherapy with mainly marfanil and noprednisone, surgery and laser therapy and so on. Conclusion Tracheobronchial amyloidosis has not special clinical characteristics and is apt to be misdiagnosed. Pathologic examination is the only measure to diagnose. Most patients are hardly cured with poor prognoses.