PMC4534991_01
Serial chest radiographs of 60-year-old female patient.. A. The Initial radiograph shows bilateral diffuse ground glass appearing density, especially in the lower lung fields.. B. Radiograph obtained one year later A shows both aggravated lower lung field lesions. Note bilateral pleural effusion and increased heart size as compared with radiograph A.
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A chest roentgenogram revealed a bilateral diffuse ground-glass density, especially in the lower lung fields, with pleural effusion (Fig. 1).
A Holter ECG recording shows sinus bradyarrhythmia with junctional escape rhythm, episode of paroxysmal supraventricular tachycardia (PSVT) and multiform ventricular premature beats (VPBs).
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A Holter ECG recording showed frequent sinus bradyarrhythmia with junctional escape rhythm, a few episodes of paroxysmal supraventricular tachycardia (sick sinus syndrome) and multiform ventricular premature beats (VPBs) (Fig. 2).
A two-dimensional echocardiogram shows a thickened left ventricular posterior wall, interatrial and interventricular septum. The myocardium shows an increased refractile pattern, a so-called "granular sparkling" appearance.
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In the echocardiogram, the left ventricular posterior wall, interventricular and interatrial septum were thickened, and the myocardium showed a hyperrefractile "granular sparkling" appearance (Fig. 3).
A high resolution CT obtained at right lower lung field (retrospective retargeting, FOV 20 cm, 140KVP, 510 mAs, 512x512 matrix, bone algorithm) shows prominent interlobular septa (white arrowhead) and a central core structure (thin arrow), as well as a diffuse, ill-defined, increased hazy density. Increased density between the rib and the lung (thick black arrows) is due to pleural effusion.
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Bilateral pleural effusion with mild pleural thickening was observed (Fig. 4).
Polarizing microscopy following Congo-red stain reveals apple-green birefringence at the vascular walls and along the alveolar septa (x400).
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Polarizing microscopy following Congo-red stain revealed apple-green birefringence at the vascular walls and along the alveolar septa (Fig. 5).
Electron microscopic observation of the lung reveals massive deposition of fibrillar material in the alveolar septum. The alveolar basement membrane (long arrows) is incorporated into the amyloid substance. Retractile white spots and fibers (tiny arrowheads) in the amyloid pool are preexisting collagen fibers. Free space (stars) in the interstitium is due to interstitial edema. Alveolar epithelial cells (P) are artifactually deformed. (E: endothelial cells; smaller arrows: capillary basement membrane). (Bar represents 1 um, x14,000). (Inset) Magnification of the amyloid substance reveals nonbranching fibrills of approximately 100 nm in length and 10 nm in diameter. Granular deposits, less than 10 nm in diameter, are scattered also. (Bar represents 0.1 um, x56,000).
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Electron microscopic observation revealed a massive deposit of fibrillar material in the alveolar septum (Fig. 6).