Yeungnam Univ J Med.  2010 Jun;27(1):63-68. 10.12701/yujm.2010.27.1.63.

A Case of Lymphangioleiomyomatosis in Lung

• Park JE
• Kim HJ
• Woo DH
• Ryu YH
• Lee KH
• Chung JH
• Shin KC

• KMID: 1462714
• DOI: http://doi.org/10.12701/yujm.2010.27.1.63

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.

Keyword

Lymphangioleiomyomatosis; Lung

MeSH Terms

Angiomyolipoma
Biopsy
Dyspnea
Female
Humans
Lipopolysaccharides
Lung
Lung Diseases
Lymphangioleiomyomatosis
Lymphatic Vessels
Myocytes, Smooth Muscle
Pneumothorax
Thorax
Tuberous Sclerosis
Venules
Lipopolysaccharides