原发性气管支气管淀粉样变的临床特征分析

Clinical features of primary tracheobronchial amyloidosis

关键词:  支气管  淀粉样变  支气管镜

Key words:  bronchi  amyloidosis  bronchoscopy

摘要:

目的　探讨原发性气管支气管淀粉样变(primary tracheobronchial amyloidosis, PTBA)患者的临床特征。　方法　收集2010年8月至2015年4月大连市中心医院呼吸内科收治的5例PTBA患者的临床资料，对患者临床表现、实验室检查、胸部CT及支气管镜下表现、治疗效果和预后进行分析与总结。　结果　3例主要临床表现为进行性气短、呼吸困难，2例为咳嗽、咳痰；1例伴有咯血，2例伴有声音嘶哑。实验室检查缺乏特异性。胸部CT及冠状位CT显示3例累及气管，1例累及气管、双侧主支气管及声门，1例累及段以下支气管。所有病例均表现为弥漫或局部管壁不规则增厚及管腔狭窄，其中伴有管壁钙化1例、肺门及纵膈淋巴结钙化1例、阻塞性肺炎1例。气管镜下可呈肿物型、管壁弥漫增厚型及坏死物堵塞管腔。5例患者行病理学检查刚果红染色均为阳性。　结论　PTBA缺乏典型的临床表现，胸部CT及纤维支气管镜是相对有效的检查手段，但临床确诊仍需要病理学检查及特殊染色。该病的治疗可根据病变的程度选择随诊观察、药物治疗及气管镜介入治疗。

Abstract:

Objective　To investigate the clinical features of primary tracheobronchial amyloidosis (PTBA).　Methods　Clinical data of 5 patients with PTBA from August 2010 to April 2015 in Dalian Municipal Central Hospital were collected.The clinical manifestations, laboratory examination, chest CT and bronchoscope under performance, treatment effect and prognosis were retrospectively analyzed and summarized.　Results　Three cases has major clinical manifestations with progressive shortness of breath and difficulty breathing, and 2 cases with cough and sputum, of which 1 case was accompanied with hemoptysis and 2 cases with hoarse voice.Laboratory tests were lack of specificity.Chest CT and coronal CT showed that 3 cases involved trachea, 1 case involved the trachea, bilateral main bronchus and the glottis, and 1 case involved the subsegmental bronchus.All cases showed diffuse or local wall irregular thickening and luminal stenosis.One of them was accompanied by tube wall calcification, one by pulmonary hilar and mediastinal lymph node calcification, and one by obstructive pneumonia.Bronchoscope examination showed the mass, wall diffuse thickening and necrosis obstructed the lumen.All cases were positive for Congo red stain by pathological examination.　Conclusion　PTBA lacks typical clinical manifestations.Chest CT and bronchoscopy are relatively effective methods, but the clinical diagnosis still needs pathological examination and special stain.According to the extent of the lesion, treatment options of the disease can be follow-up, drug therapy or bronchoscopy interventional therapy.