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Nodular Amyloidosis Presenting as Acute Respiratory Failure Secondary to Massive Hemoptysis and Concurrent Hemothorax FREE TO VIEW

Kusum Mathews*, MD; Mario Katigbak, MD; Aydin Uzunpinar, MD
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Yale School of Medicine, New Haven, CT

Chest. 2012;142(4_MeetingAbstracts):968A. doi:10.1378/chest.1381802
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SESSION TYPE: Miscellaneous Case Report Posters I

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Amyloidosis can involve lung parenchyma in a diffuse, tracheobronchial, or parenchymal pattern and can be associated with pulmonary hemorrhage. We present a case of nodular parenchymal amyloidosis presenting with both hemoptysis and spontaneous hemothorax.

CASE PRESENTATION: A 78 year-old woman with breast and renal amyloidosis, presented with acute abdominal pain. She developed acute hematemesis, requiring intubation for hypoxemic respiratory failure. Frank blood was noted in the endotracheal and orogastric tubes. Her hematocrit continued to decrease despite aggressive transfusion. Chest film, previously indicative of limited aspiration event, showed full opacification of right hemithorax. Emergent EGD revealed small amount of clotted blood, but no source of hematemesis. Suspecting a pulmonary etiology, emergent bronchoscopy was performed and revealed fresh blood arising from the right lower lobe. Bronchial angiography did not visualize active hemorrhage, but the right bronchial artery was embolized empirically. Fluoroscopy images during the angiogram indicated a layering right pleural effusion. A chest tube was placed at bedside with drainage of 1600cc of blood. CT Chest after stabilization revealed residual effusion, multiple parenchymal consolidations, and nodular densities (Fig. 1). She later underwent VATS-guided wedge biopsy. Lung tissue appeared boggy due to retained hemorrhage, visceral pleura showed nodularity, and an area of pleural rupture along the upper lobe’s inferior surface was visualized. Pathology revealed a subpleural nodule with positive green birefringence on Congo Red stain, surrounded by intraalveolar hemorrhage (Fig. 2).

DISCUSSION: Nodular pulmonary amyloidosis is a rare occurrence, usually affecting older patients. It can appear as a peripheral circumscribed mass or multiple nodules on imaging. The amyloid can infiltrate vascular walls leading to vessel fragility and impaired vasoconstriction, deposit into bronchi causing bronchiectasis, and is associated with coagulopathy. Amyloidosis with diffuse alveolar hemorrhage and hemoptysis has been seen in case reports, but only one report linked this disease with resulting hemothorax. This is an unusual case of both hemoptysis and hemothorax as presenting symptoms in a patient with nodular pulmonary amyloidosis. This patient’s hemothorax likely resulted from spontaneous rupture of visceral pleura in an area with amyloid nodule-induced alveolar hemorrhage.

CONCLUSIONS: Nodular pulmonary amyloidosis has been associated with bronchiectasis, hemotypsis, and vascular infiltration. As seen in our case, the associated vessel fragility can lead to extensive pulmonary hemorrhage and when involving visceral pleura, can result in hemothorax.

1) Yood et al. Bleeding manifestations in 100 patients with amyloidosis. JAMA 1983;249:1322-4.

2) Alwitry et al. Vascular amyloidosis causing spontaneous mediastinal haemorrhage with haemothorax. European Journal of Cardiothoracic Surgery 2001;4;871-3.

DISCLOSURE: The following authors have nothing to disclose: Kusum Mathews, Mario Katigbak, Aydin Uzunpinar

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Yale School of Medicine, New Haven, CT




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