Linfangioleiomiomatosis pulmonar: Caso clínico

Silva O, Rafael; Puelma C, Felipe; Retamal P, Víctor; Rojas SM, Pedro; Cruzat C, Claudio; Reyes G, Cecilia

Keywords: lung diseases, pneumothorax, lymphangioleiomyomatosis

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).

Más información

Título según SCIELO: Linfangioleiomiomatosis pulmonar: Caso cl�nico
Título de la Revista: REVISTA MEDICA DE CHILE
Volumen: 137
Número: 11
Editorial: SOC MEDICA SANTIAGO
Fecha de publicación: 2009
Página de inicio: 1474
Página final: 1477
Idioma: es
URL: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009001100010&lng=en&nrm=iso&tlng=en
DOI:

10.4067/S0034-98872009001100010

Notas: SCIELO