[Lymphangioleiomyomatosis: a case report].
Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology affecting woman, primarily in their reproductive years. It is characterized by non-neoplasic proliferation of atypical smooth muscle cells that may lead to airway, lymphatic and blood vessel obstruction over time.A nine-year followed-up case of LAM is described,which presents with some unusual details as worsening haemoptysis in the setting of pregnancy and absence of cough or pneumothorax along the course of the disease. Neither haemoptysis nor gradual pulmonary function deterioration seemed to be influenced by hormone therapy. A brief re-view of the disease is then presented.
Costa
Antonio Santos
AS
Exames Especiais de Pneumo;ogia, Centro Hospitalar de Vila Real/Peso da Reguna. Amsantoscosta@gmail.com
Noya
Rafael
R
Calvo
Teresa Campos
TC
Severo
R
R
Afonso
Abel
A
D000328:Adult; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis*; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis*; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis*