Pulmonary Lymphangioleiomyomatosis: A Rare, Diffuse Parenchymal Lung Disease
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Case report
VOLUME: 12 ISSUE: 3
P: 124 - 126
September 2011

Pulmonary Lymphangioleiomyomatosis: A Rare, Diffuse Parenchymal Lung Disease

Turk Thorac J 2011;12(3):124-126
1. Yedikule Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, 3. Göğüs Cerrahisi Kliniği, Göğüs Cerrahisi, İstanbul, Türkiye
2. Yedikule Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, 3. Göğüs Hastalıkları Kliniği, İstanbul, Türkiye
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Accepted Date: 18.07.2019
Online Date: 18.07.2019
Publish Date: 18.07.2019
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Abstract

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting fertile women and is characterized by progressive dyspnea on exertion. Clinically, it could also be characterized by recurrent pneumothorax, haemoptysis or chylous pleural effusions. It is characterized by peribronchial, perivascular, and perilymphatic proliferation of atypical smooth muscle, resulting in vascular and airway obstruction, cyst formation, and a progressive decline in lung function. The diagnosis of LAM usually requires an open lung biopsy. Here we presented 3 cases that were diagnosed as LAM by surgical pathology. (Tur Toraks Der 2011; 12: 124-6)

Keywords:
Lymphangioleiomyomatosis, diagnosis, pneumothorax, dyspnea