SESSION TITLE: Pleural Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 28, 2013 at 04:15 PM - 05:15 PM
INTRODUCTION: Hemothorax is defined by a pleural fluid hematocrit greater than 50% of the blood hematocrit. Most are traumatic in origin. Spontaneous hemothorax is much less common and can have a variety of causes. We present a novel case of spontaneous hemothorax as a result of pulmonary amyloidosis.
CASE PRESENTATION: This is a 51-year-old female with a two-year history of interstitial lung disease of unknown etiology, who presented with sudden onset dyspnea and hemoptysis. Vital signs were normal except for moderate hypoxemia. Physical exam was remarkable for bibasilar inspiratory crackles and diminished breath sounds over the left base. Chest CT angiography was negative for thromboembolism, but did reveal multi-lobar interlobular septal thickening, patchy ground glass changes, and left lower lobe consolidation. Bronchoscopy showed bright red blood throughout the left lower lobe segmental and subsegmental bronchi. Bronchoalveolar lavage cultures grew oropharyngeal microbes. No bronchoscopic biopsies were attempted. A left pleural effusion subsequently developed, and a thoracentesis was performed. The pleural fluid was frankly bloody with a hematocrit of 16.4% (blood hematocrit: 30.8%). Cytology was negative. She subsequently underwent open thoracotomy. Intraoperatively, a large amount of clotted blood was evacuated from the pleural space. The lung itself was brittle and appeared to be fracturing in multiple places. Wedge biopsies revealed diffuse involvement of the lung parenchyma and vasculature by AL amyloidosis. Further evaluation discovered a monoclonal gammopathy, bone marrow involvement, and cardiac infiltration. She was started on lenalidomide and dexamethasone. There was no recurrence of hemothorax post-operatively.
DISCUSSION: Hemothorax is commonly seen after blunt or penetrating trauma. Spontaneous hemothorax is uncommon with the majority caused by invasive neoplasms, pleural metastasis, coagulopathy, or pulmonary infarction. Pulmonary amyloidosis has not been previously reported to cause spontaneous hemothorax. We hypothesize that the pathogenesis is related to amyloid angiopathy. Fibril deposition within blood vessel walls results in increased vascular fragility and can lead to spontaneous rupture or dissection. Amyloid angiopathy has been previously reported to cause hemoptysis, pulmonary infarction, hematoma, or arteriovenous fistula formation. Development of hemothorax in this case was likely due to bleeding from spontaneous rupture of pleural-based vessels and/or pulmonary infarction. Amyloid angiopathy likely caused both hemothorax and hemoptysis in our patient.
CONCLUSIONS: Pulmonary amyloidosis is a rare cause of spontaneous hemothorax. The pathogenesis is likely related to amyloid angiopathy.
Reference #1: Ali, HA et al. Spontaneous Hemothorax. Chest. 2008; 134: 1056-65
Reference #2: Cordier, JF. Pulmonary Amyloidosis and Hematologic Disorders. Seminars in Respiratory and Critical Care Medicine. 2005; 26: 502-513
DISCLOSURE: The following authors have nothing to disclose: Jim Liu, Douglas Closser, Anjali Satoskar, Jerome Stasek
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