Amyloidosis is a disorder of extracellular protein deposition that encompasses a wide spectrum of related diseases. The underlying pathology involves abnormal protein folding and results in insoluble amyloid fibril proteins accumulating in normal tissues. Various subtypes exist, including primary systemic amyloidosis, reactive systemic amyloidosis associated with chronic inflammatory states, and localized forms of aberrant amyloid deposition.
Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways and is typically associated with monoclonal Ig light chains.2
The clinical effects of TBA include dyspnea, cough, hemoptysis, and hoarseness. Pulmonary function abnormalities depend on the location of involvement with proximal deposition typically causing obstructive physiology and mid/distal disease demonstrating normal airflow rates and air trapping.
TBA has typically been managed by bronchoscopic methods including mechanical debridement, laser ablation, balloon dilation, and stent placement. Local excisions often prove temporarily effective; however, multiple local recurrences with progressive compromise of pulmonary function may occur, often leading to death. External beam radiation therapy has been successfully utilized in a limited number of case reports.” We present a case of a patient with localized TBA in which symptomatic, radiographic, and bronchoscopic observation demonstrated improvement with localized external beam radiation therapy as the primary treatment modality. avandia generic
The patient is a 60-year-old white woman with multiple medical comorbidities, including COPD, hypertension, diabetes mellitus, coronary artery disease, congestive heart failure, and an 80-pack-a-year history of cigarette smoking. She presented in June 2001 to an outside institution with a 3-week history of increasing shortness of breath, cough productive of white sputum, and occasional hemoptysis. The patient required 4 L/min of oxygen by nasal cannula at rest to maintain adequate oxygenation. A chest radiograph obtained at hospital admission was significant for collapse of the right lower lobe and a right pleural effusion.