Source: Annual Congress 2008 - Mediastinal lesions; rare pathology
Disease area: Interstitial lung diseases

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare cause of recurrent pneumothorax and should be considered a differential diagnosis, especially in young women with bilateral bullous emphysema.
We present two women (54 and 36-years-old) with bilateral successive recurrent spontaneous pneumothorax and hemoptysis, treated by open thoracothomy (first case) and video-assisted thoracic surgery (second case). Bullae and blebs were resected by stapler and vigorous pleural abrasion was carried out. Vanderschueren staging was stage II in first case and stage III in second case.The functional respiratory status was normal in the young patient, but the 54 years-old woman has an obstructive spirometric disfunction. The thoracic computed tomography was abnormal in one patient with rare air filled cysts in bilateral lung parenchyma.
The diagnosis of LAM was established retrospectively, during a imunohistochemical study in lung biopsy specimens of operated patients with primary spontaneous pneumothorax.
Both cases had LAM cells in the lung parenchyma with receptors for oestrogen and progesterone, anti-smooth muscle-actine positive cells.Monoclonal antibody for melanoma related antigen (HMB45) positive cells were present only in the young woman.
In conclusions LAM is a rare lung disorder, characterized by abnormal proliferation of smooth muscle and cystic air spaces and should be suspected in young women who have dyspnea, hemoptysis, cough and interstitial radiological pattern, especially when is associated with a recurrent spontaneous pneumothorax.

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Citations should be made in the following way:
C. Grigorescu, M. L. Bosanceanu, L. G. Cosma (Iasi, Romania). Pulmonary lymphangioleiomyomatosis – a rare cause of bilateral recurrent spontaneous pneumothorax. Eur Respir J 2008; 32: Suppl. 52, 2151

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