Sudden Onset of Dyspnoea and Chest Pain in a 39-Year-Old Woman

2002 
Accessible online at: www.karger.com/journals/res A 39-year-old previously healthy nurse presented with a 2-week history of chest pain with sudden onset and dyspnoea grade II. The never-smoker was on no medication but reported recurring episodes of self-limited non-productive cough since puberty. On examination the patient appeared healthy. Reduced breath sounds over the right hemithorax were noted. The remainder of the examination was within normal limits. Routine laboratory examinations were normal. The chest X-ray (fig. 1) showed a right-sided pneumothorax and diffuse interstitial infiltrates. Initial intercostal tube drainage was unsuccessful so that a high resolution CT scan was performed (fig. 2, slice at the lung base) which showed bilateral cystic lesions throughout the lung fields suggestive of pulmonary lymphangioleiomyomatosis (LAM). Although the latter examination is usually considered diagnostic for LAM the patient underwent confirmatory open lung biopsy as surgical pleurectomy was planned to treat the non-resolving pneumothorax. Multiple ‘blebs’ of the visceral pleura and a very inflamed parietal pleura were seen intra-operatively. The histology showed cysts with abnormal proliferation of smooth muscle cells (LAM cells, arrow in fig. 3 ). HMB-45, a highly sensitive and specific immunohistological marker for LAM, was positive.
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