SESSION TITLE: Miscellaneous Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Wednesday, October 28, 2015 at 11:00 AM - 12:15 PM
INTRODUCTION: Amyloidosis is an uncommon disease, which affects multiple body organs. Localized forms of amyloidosis can involve the lung in various ways. Of these,tracheobronchial amyloidosis (TBA) is the most common but still a rare entity.TBA has diverse manifestations and may present insidiously.This may lead to misdiagnosis and may delay the diagnosis in the presence of other chronic respiratory diseases.
CASE PRESENTATION: We describe two patients with isolated TBA. The patients were male and were 47 and 62 years old. They are both presented with progressive shortness of breath and cough. Chest x-ray demonstrated right upper lobe (RUL) collapse and left upper lobe (LUL) collapse respectively that was confirmed by computed tomography scan(image-1). Bronchoscopy showed endobronchial lesions in both patients (image-2). Pathologies were consistent with amyloid light chain amyloidosis. Systemic amyloidosis was excluded in both patients after extensive investigations. The first patient underwent right upper lobectomy after unsuccessful laser ablation. He refused further invasive managements and his disease has progressed slowly over 20 years.The second patient was observed closely after an unsuccessful trial of argon plasma coagulation and local resection.
DISCUSSION: Tracheobronchial amyloidosis results from amyloid deposition from plasma cells surrounding the airway. It does not occur in systemic amyloid. It can produce submucosal plaques or tumor like masses, which may be localized, diffuse or multifocal. Its clinical presentations depend on the anatomical distribution1.There are three identified patterns of involvement: proximal, mid-bronchial or distal bronchial tree. Both of our cases presented initially with evidence of localized lung collapse. In one patient, amyloidosis has progressed to involve the proximal trachea and subglottic area.
CONCLUSIONS: Although TBA is a localized disease process, the course may not be benign and varies according to the degree of involvement. Different treatment modalities have been attempted such as bronchoscopic debridement and debulking, external beam radiation therapy and laser ablation, but there is currently no fully effective treatment. Herein we describe two cases of TBA with differing presentations, to draw attention to this uncommon entity, its clinical presentation, and available management.
Reference #1: Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis.The Mayo Clinic experience from 1980 to 1993. Annals of internal medicine.1996;124(4):407-13.Epub1996/02/15.PubMed PMID:8554249
DISCLOSURE: The following authors have nothing to disclose: Sultan Qanash, Nadir Kharma, Stephen Corne
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