Abstract

VALENTE, Carla et al. Lymphangioleiomyomatosis - report of three cases. Rev Port Pneumol [online]. 2010, vol.16, n.1, pp.187-196. ISSN 0873-2159.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology. It is characterized by proliferation of abnormal smooth-muscle cells throughout the peribronchial, perivascular and perilymphatic regions of the lung. LAM may occur sporadically, in association with tuberous sclerosis complex (TSC) or inheritable multiorgan hamartomatosis. In either situation, LAM occurs almost exclusively in women of reproductive age, and approximately one third of the patients with TSC have LAM. The authors review the cases of three female patients diagnosed with LAM based on clinical and radiological findings. A brief review of the disease is then presented.

Keywords : Lymphangioleiomyomatosis; tuberoussclerosis complex.

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