A Case of Lymphangioleiomyomatosis in Lung.
10.12701/yujm.2010.27.1.63
- Author:
Jung Eun PARK
1
;
Hyun Jung KIM
;
Dae Hyung WOO
;
Yung Ha RYU
;
Kwan Ho LEE
;
Jin Hong CHUNG
;
Kyeong Cheol SHIN
Author Information
1. Department of Internal Medicine, College of Medicine, Yeungnam University, Daegu, Korea. shin@med.yu.ac.kr
- Publication Type:Case Report
- Keywords:
Lymphangioleiomyomatosis;
Lung
- MeSH:
Angiomyolipoma;
Biopsy;
Dyspnea;
Female;
Humans;
Lipopolysaccharides;
Lung;
Lung Diseases;
Lymphangioleiomyomatosis;
Lymphatic Vessels;
Myocytes, Smooth Muscle;
Pneumothorax;
Thorax;
Tuberous Sclerosis;
Venules
- From:Yeungnam University Journal of Medicine
2010;27(1):63-68
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutation in the tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread and a remarkable female gender predilection. The pathology of LAM is represented by the proliferation of immature smooth muscle cells in the walls of airways, and venules and lymphatic vessels in the lung. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax and collections of chylous fluid. The diagnosis of pulmonary LAM can be made on chest X-ray, a high-resolution CT scan and lung biopsy. We experienced a case of pulmonary lymphangioleiomyomatosis in a 28-years-old female patient who had suffered from progressive dyspnea on exertion, so we report on it along with a brief review of the relevant literature.
