Pulmonary lymphangioleiomyomatosis: report of one case

Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease, of unknown etiology, affecting almost exclusively women. Microscopically LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other diseases (sporadic LAM) or in conjunction with tuberous...

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Bibliographic Details
Published in:Revista medíca de Chile 2009-11, Vol.137 (11), p.1474-1477
Main Authors: Silva O, Rafael, Puelma C, Felipe, Retamal P, Víctor, Rojas Sm, Pedro, Cruzat C, Claudio, Reyes G, Cecilia
Format: Article
Language:Spanish
Subjects:
Adult
Antibodies, Monoclonal - analysis
Female
Humans
Lung Diseases, Interstitial - pathology
Lung Neoplasms - complications
Lung Neoplasms - pathology
Lymphangioleiomyomatosis - complications
Lymphangioleiomyomatosis - pathology
Pneumothorax - etiology
Recurrence
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Summary:Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease, of unknown etiology, affecting almost exclusively women. Microscopically LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other diseases (sporadic LAM) or in conjunction with tuberous sclerosis complex (TSC). It presents with progressive breathlessness or with recurrent pneumothorax or chylothorax. We report a 33 year-old woman with a history of recurrent pneumothorax. Computed tomography (CT) scans showed numerous thin-walled cysts throughout the lungs, a characteristic finding in LAM. A pulmonary biopsy was compatible with the diagnosis and HMB-45 monoclonal antibodies were positive. Treatment with Sirolimus was started).
ISSN:0034-9887