Lymphangioleiomyomatosis (LAM)--an uncommon cause of bilateral spontaneous pneumothorax

Lymphangioleiomyomatosis (LAM)--an uncommon cause of bilateral spontaneous pneumothorax

Rev Med Chir Soc Med Nat Iasi. 2007

Grigorescu C, Bosânceanu M, Boişteanu D, Aldea A, Chiseliţă I, Cozma LG.
Clinica de Chirurgie Toracică, Facultatea de Medicină, Universitatea de Medicină si Farmacie "Gr. T. Popa", Iaşi.

LAM, a rare lung disease typically affecting women of reproductive age, is characterized by abnormal proliferation of smooth--muscle cells and progressive loss of pulmonary function due to destruction of lung parenchyma. Two cases of bilateral succesive recurrent spontaneous pneumothorax and haemoptysis are presented.

Repeated conventional and video-assisted surgery was required in both cases, for drainage of the recurrent pneumothorax and resection of subpleural bulae, with good immediate postoperative evolution. Immunohistochemical studies of resected specimens revealed LAM cells in the lung parenchyma with receptors for oestrogen and progesterone. HMB45 monoclonal antibodies in the LAM cells were identified in one case.

The follow-up of the patients revealed no signs of recurrence at 84 and 18 months respectively, although pulmonary transplantation should be considered in case of further deterioration of respiratory function.

PMID: 17595857 [PubMed - in process]