Tuberc Respir Dis.  2008 Dec;65(6):532-536.

A Case of Primary Localized Laryngo-tracheobronchial Amyloidosis

• Lee SJ
• Lee WY
• Jung SH
• Kwon W
• Lee SN
• Lee N
• Kim SH
• Shin KC
• Yong SJ

Abstract

We report on a case of a patient with laryngo-tracheobronchial amyloidosis who complained of cough, sputum, and hoarseness. A chest X-ray showed consolidation in the right middle lobe. A chest CT scan showed diffuse, irregular narrowing of the tracheobronchial tree and atelectasis of the right middle lobe, with calcification of bronchial wall. Bronchoscopic findings were multinodular submucosal thickening of the right vocal cord, and yellowish multinodular submucosal thickening from the lower trachea through both main bronchi, as well as the lingular division of the left upper lobe, the right middle lobe, and the right lower lobe. The right middle lobe bronchus was nearly obstructed. The diagnosis of amyloidosis was made by multiple bronchoscopic biopsies on the right vocal cord and both bronchi. Pathologic findings were characteristic apple-green birefringence under polarized microscopy with Congo-red stain. The patient had no evidence of systemic amyloidosis. The patient is under conservative symptomatic treatment.

Keyword

Tracheobronchial amyloidosis; Laryngeal amyloidosis; Right middle lobe syndrome; Hoarseness

MeSH Terms

Amyloidosis
Biopsy
Birefringence
Bronchi
Cough
Hoarseness
Humans
Microscopy
Pulmonary Atelectasis
Sputum
Thorax
Trachea
Vocal Cords

Figure

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  Figure 1 Chest X-rays. Consolidation was revealed in the right middle lobe. Trachea was diffusely and irregularly narrowed (arrow).

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  Figure 2 Chest CT. Tracheobronchial tree was diffusely and irregularly narrowed. Atelectasis of the right middle lobe and calcification of bronchial wall were revealed.

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  Figure 3 Bronchoscopy. (A) Multinodular submucosal thickening revealed in the right false and true vocal cord. (B) Yellowish multinodular submucosal thickening was revealed from lower trachea to both main bronchi. (C) The right middle lobe bronchus was nearly obstructed.

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  Figure 4 Pathologic findings. (A) Light microscopic finding was diffuse deposition of eosinophilic amorphous material in the submucosa (H&E stain, ×200). (B) The Congo-red stain for amyloid revealed characteristic apple green-colored birefringency under the polarized microscope (×200).

Reference

1. Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis: the Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996. 124:407–413.

2. Gibbaoui H, Abouchacra S, Yaman M. A case of primary diffuse tracheobronchial amyloidosis. Ann Thorac Surg. 2004. 77:1832–1834.

3. Kwon SW, Kim YK, Jung KH, Kim DS, Jeon WK, Suh YL. A case of tracheo-bronchial amyloidosis. Korean J Med. 1993. 45:690–695.

4. Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax. 1999. 54:444–451.

5. Yamamoto T, Maeda M, Mizuno S, Saga T, Itoh H. Primary diffuse tracheobronchial amyloidosis: radiologic findings. J Thorac Imaging. 2001. 16:177–180.

6. Dahl KA, Kernstine KH, Vannatta TL, Karwal MW, Thomas KW, Schraith DF. Tracheobronchial amyloidosis: a surgical disease with long-term consequences. J Thorac Cardiovasc Surg. 2004. 128:789–792.

7. Kurtz KA, Kirby PA. Pathologic quiz case: a 49-year-old man with chronic cough and a left lung hilar mass. Tracheobronchial amyloidosis. Arch Pathol Lab Med. 2003. 127:e420–e422.

8. Piazza C, Cavaliere S, Foccoli P, Toninelli C, Bolzoni A, Peretti G. Endoscopic management of laryngo-tracheobronchial amyloidosis: a series of 32 patients. Eur Arch Otorhinolaryngol. 2003. 260:349–354.

9. O'Regan A, Fenlon HM, Beamis JF Jr, Steele MP, Skinner M, Berk JL. Tracheobronchial amyloidosis: the Boston University experience from 1984 to 1999. Medicine (Baltimore). 2000. 79:69–79.

10. Cho CW, Kim BK, Choi SY, Lee TH, Kim MA, Kang DS, et al. A case of pseudotumorial tracheabronchial amyloidosis. Korean J Med. 1995. 48:816–821.

11. Kim MO, Sohn JW, Yang SC, Yoon HJ, Shin DH, Park SS, et al. A case of tracheobronchial amyloidosis treated by laser therapy. Korean J Med. 2003. 65:350–354.

12. Kwak YG, Kim HJ, Lee CH, Kim SY, Cho JH, Kwak SM, et al. A case of primary localized tracheobronchial amyloidosis. Turbec Respir Dis. 2002. 52:174–178.

13. Shah PL, Gillmore JD, Copley SJ, Collins JV, Wells AU, du Bois RM, et al. The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract. Sarcoidosis Vasc Diffuse Lung Dis. 2002. 19:134–142.

14. Kim HJ, Koh JH, Chang MJ, Hong SH, Kim KH, Hyun IK, et al. Treatment of diffuse tracheobronchial amyloidosis by repeated electrocautry under fiberoptic bronchoscopy. Turbec Respir Dis. 1995. 42:250–255.

15. Monroe AT, Walia R, Zlotecki RA, Jantz MA. Tracheobronchial amyloidosis: a case report of successful treatment with external beam radiation therapy. Chest. 2004. 125:784–789.