[Pulmonary lymphangioleiomyomatosis. A disease which may have a benign progression].
Pulmonary lymphangioleiomyomatosis (PLLM) is characterized by a benign proliferation of the smooth muscle around lymphatics, venulas and bronchiolus. It is usually fatal within 1 to 9 years. We present a case which remained with scarce symptoms despite receiving no treatment after at least 9 years of evolution of the disease. We highlight the clinical manifestations of the disease and the relevance of pulmonary biopsia with thoracotomy in order to establish the diagnosis.
Servicio de Neumología, Hospital General, Valencia.
M J
MJ
Barranco Simó
M A
MA
Ciscar Vilanova
J L
JL
Ibor Otamendi
J
J
Ferrando Marco
G
G
Juan Samper
D000328:Adult; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000503:physiopathology*; D008203:Lymphangiomyoma / Q000175:diagnosis / Q000503:physiopathology*
Linfangioleiomiomatosis pulmonar. Una enfermedad que puede tener un curso benigno.