Linfangioleiomiomatosis pulmonar: Caso clínico / Pulmonary lymphangioleiomyomatosis: Report of one case
Rev. méd. Chile
;
137(11): 1474-1477, nov. 2009. ilus
Article
in Spanish
| LILACS
| ID: lil-537011
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Lymphangioleiomyomatosis
/
Lung Diseases, Interstitial
/
Lung Neoplasms
Type of study:
Etiology study
Limits:
Adult
/
Female
/
Humans
Language:
Spanish
Journal:
Rev. méd. Chile
Journal subject:
Medicine
Year:
2009
Type:
Article
Affiliation country:
Chile
Institution/Affiliation country:
Hospital Base de Linares/CL
/
Hospital Regional de Talca/CL
/
Universidad de Talca/CL
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