气管支气管淀粉样变7例临床分析  

Clinical analysis of seven cases of tracheobronchial amyloidosis

作　　者：张予辉[1] 黄克武[1] 陈文慧[1] 梁荣章[2] 郭丽娟[1] 

机构地区：[1]首都医科大学附属北京朝阳医院北京呼吸疾病研究所,100020 [2]福建医科大学附属医院龙岩市第一医院呼吸内科,364000

出　　处：《国际呼吸杂志》2010年第19期1161-1164,共4页International Journal of Respiration

摘　　要：目的探讨气管支气管淀粉样变（tracheobronchial amyloidosis，TBA）患者的临床特征和预后。方法回顾性分析1999年6月至2009年2月首都医科大学附属北京朝阳医院收治的7例TBA患者的临床资料，并结合文献进行分析。结果7例患者中男5例，女2例；年龄33～71岁，平均年龄52．9岁。主要临床表现为反复咳嗽（6／7）、咳痰（6／7）、喘憋（5／7）、咯血（3／7）、发热（2／7）及声嘶（3／7）。7例患者中5例胸片无明显异常；但7例患者胸部CT均可见受累气管支气管壁增厚、管腔不规则狭窄，2例伴管壁钙化，1例伴阻塞性肺炎和肺不张改变。7例患者均经支气管镜检查和取组织活检确诊。病理学检查7例患者支气管黏膜可见均质嗜伊红物质沉积，刚果红染色阳性。6例患者行肺功能检查，以中重度阻塞性通气功能障碍为主要表现，弥散量轻度降低或正常。目前治疗主要是减轻症状，可选用经支气管镜行介入治疗、手术或放疗等。7例患者随访时间为7个月～10年，1例老年、病变广泛且有心功能不全的患者死亡，3例病变累及气管的患者缓慢进展，3例患者稳定。结论TBA是少见的缓慢进展的疾病，临床表现取决于气道受累的部位和程度，目前无确切治疗方法，气管受累的患者预后差。Objective To analyze the clinical features and prognosis of patients with tracheobronchial amyloidosis（TBA）. Methods Clinical data of 7 TBA patients collected from June 1999 to February 2009 were reviewed and analyzed together with literature. Results There were 5 male and 2 female patients, aged from 33 years to 71 years （mean age was 52. 9 years）. The main clinical manifestations included chronic cough （6/7）, sputum （6/7）, short breath （ 5/7 ）, hemoptysis （2/7）, fever （2/7） and hoarseness （3/7）. Chest X-rays of 5 out of 7 patients were normal. But chest CT of 7 patients showed irregular luminal narrowing and airway wall thickening, specially 2 of 7 showed calcification of airway wall,and 1 companied with obstructive pneumonia and atelectasis. All of 7 cases were diagnosed through bronchoscopes along with biopsy-proven TBA. Pathologic examination showed 7 cases had homogeneous addict hemagglutinin-esterase substance deposition in bronchial wall by confirmation of amyloid with Congo red histology. Further pulmonary function testing of 6 patients revealed obstructive ventilatory disturbance, and diffusion capacity for carbon monoxide were almost normal or reduced slightly. So far the treatments of TBA mainly alleviated the symptoms adopting interventional therapy through bronchoscopes,surgery and local radiotherapy. Follow-up ranging from 7 month to 10 years found that 1 senior patient with diffuse lesion and heart insufficiency was dead, 3 cases with lesion involving trachea had progress slowly, and the other 3 cases remained stable. Conclusions TBA was a rare and slowly progressive disease, and its clinical manifestations depended on the airway lesion of location and extent. No well-recognized therapy has been achieved yet. The cases with lesion involving trachea had poor prognosis.

关 键 词：淀粉样变 支气管 临床特征 预后 

分 类 号：R5[医药卫生—内科学]