Linfangioleiomiomatosis pulmonar: Caso clínico / Pulmonary lymphangioleiomyomatosis: Report of one case
Rev. méd. Chile
; 137(11): 1474-1477, nov. 2009. ilus
Article
em Es
| LILACS
| ID: lil-537011
Biblioteca responsável:
BR1.1
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Índice:
LILACS
Assunto principal:
Linfangioleiomiomatose
/
Doenças Pulmonares Intersticiais
/
Neoplasias Pulmonares
Tipo de estudo:
Etiology_studies
Limite:
Adult
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Female
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Humans
Idioma:
Es
Revista:
Rev. méd. Chile
Assunto da revista:
MEDICINA
Ano de publicação:
2009
Tipo de documento:
Article