Linfangioleiomiomatosis pulmonar: Caso clínico

• Rafael Silva O ^[1] ; Felipe Puelma C ^[1] ; Víctor Retamal P ^[3] ; Pedro Rojas SM ^[1] ; Claudio Cruzat C ^[2] ; Cecilia Reyes G ^[1]
  1. [1] Hospital Regional de Talca

     Hospital Regional de Talca

     Provincia de Talca, Chile

     
  2. [2] Universidad de Talca

     Universidad de Talca

     Provincia de Talca, Chile

     
  3. [3] Hospital Base de Linares Servicio de Radiología

  Mostrar afiliaciones +
• Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 137, Nº. 11, 2009
• Idioma: español
• Títulos paralelos:
  • Pulmonary lymphangioleiomyomatosis: Report of one case
• Enlaces
  • Texto completo
• Dialnet Métricas: 1 Cita
• Resumen

  • 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).