[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.
Barranco Simó
M J
MJ
Servicio de Neumología, Hospital General, Valencia.
Ciscar Vilanova
M A
MA
Ibor Otamendi
J L
JL
Ferrando Marco
J
J
Juan Samper
G
G
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.