Clinical analysis of seven cases of tracheobronchial amyloidosis

Abstract

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 2female 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. Key words: Amyloidosis; Bronchi; Clinical features; Prognosis