A Case of Tracheobronchial Amyloidosis Treated with Endoscopic Debulking and External Beam Radiation Therapy Therapie einer tracheobronchialen Amyloidose mit endoskopischer Rekanalisation und Bestrahlung

نویسندگان

  • U. Setinek
  • H. Koller
  • P. Feurstein
  • H. Prosch
  • O. C. Burghuber
چکیده

We report on a 55-year-old man of Turkish descent whowas admitted to hospital because of recurrent pneumonia. Besides a smoking history of 30 pack-years his past medical history was unremarkable. Physical examination was entirely unremarkable. Spirometry demonstrated an (postbronchodilator) airway obstruction with a FEV1 of 1,27L (38% predicted), FVC 2,83L (71% predicted), and FEV1/FVC ratio of 54%. Bodyplethysmographydisclosed normal total lung capacity (6,03L, 107% predicted) and a residual volume of 3,2L. Laboratory results were normal. A chest CTscan revealed extensive irregular thickening of the walls of the right and left main bronchi, as well as the lobar and segmental bronchi on both sides with some calcifications. The thickening of the walls of the bronchi led to a significant narrowing of the right upper lobe and the lower lobe bronchi on both sides. Videobronchoscopy demonstrated polypoid, toric-shaped changes of the distal tracheal wall spreading into both the left and right bronchial system up to the 4th generation of bronchi. The observed bronchial abnormalities were leading to multilevel obstruction of the central airways bilaterally (●" Fig.1a,1b). Biopsies were taken. The microscopic work-up showed amorphous eosinophilic deposits in the bronchial walls. Congored staining revealed the characteristic applegreen birefringence of the amyloid deposits by

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تاریخ انتشار 2013