原发性气管支气管淀粉样变的临床及病理学观察  

Clinical and pathological observation of primary tracheobronchial amyloidosis

作　　者：王承志[1] 王咏梅[1] 杨庆婵 WANG Chengzhi;WANG Yongmei;YANG Qingchan(Department of Pathology,Tianjin Haihe Hospital/Tianjin Institule for Respiratory Diseases,Tianjin 300350,China)

机构地区：[1]天津市海河医院/天津市呼吸疾病研究所病理科,300350

出　　处：《重庆医学》2020年第22期3759-3762,共4页Chongqing medicine

摘　　要：目的探讨原发性气管支气管淀粉样变(PTBA)的临床和病理学特征。方法对该院2009年1月至2019年12月收治13例PTBA患者的临床表现、影像学、病理学及治疗预后等资料进行回顾性分析。结果PTBA患者临床表现常为咳嗽、咳痰、发热、咯血及喘息。影像学胸部CT显示患者支气管管壁增厚、狭窄,气管内结节,部分伴有钙化。苏木素-伊红(HE)染色显微镜下显示支气管黏膜内可见均质粉染的无定形物质沉积,刚果红染色阳性。PTBA以局部治疗为主,常采用支气管镜下气道消融术,缓解气道的梗阻。该组2例患者失访,其余11例患者随访6~72个月,死亡3例,3例患者病情反复,5例患者症状改善。结论PTBA的临床表现及影像学特征缺乏特异性,常延误患者的诊治,支气管镜检查是诊断该病的主要方式,确诊有赖于病理组织学及刚果红染色。Objective To investigate the clinical and pathological features of primary tracheobronchial amyloidosis(PTBA).Methods The clinical manifestations,imaging,pathology and prognosis of 13 patients with PTBA were retrospectively analysed.Results The clinical manifestations of patients with PTBA were cough,expectoration,fever,hemoptysis and wheezing.Imaging chest CT showed that bronchial wall thickening and bronchial narrowing,tracheal nodules and partial calcification.Hematoxylin-Eosin(HE)staining showed that homogeneous powder-stained amorphous substance was deposited in the bronchial mucosa,and Congo red staining was positive.Local treatment was the main treatment for PTBA,and bronchoscopic airway ablation techniques were commonly used to relieve airway obstruction.Two patients in this group were lost to follow-up,and the other 11 patients were followed up for 6 to 72 months.Among them,three patients died,three patients relapsed,and 5 patients improved.Conclusion The clinical manifestations and imaging features are lack of specificity for diagnosing PTBA,which often delay the diagnosis and treatment.Bronchoscopy is the main way to diagnose PTBA,and the diagnosis depends on histopathology and Congo red staining.

关 键 词：气管支气管病变 淀粉样变性 病理学 临床特征 治疗结果 

分 类 号：R562[医药卫生—呼吸系统]