A Case of Lymphangioleiomyomatosis Combined with Chylothorax and Bilateral Pneumothoraces.

Kim, Jong Hwa; Kim, Yang Ki; Kim, Jung Hyun; Lee, Young Mok; Kim, Ki Up; Uh, Soo Taek; Noh, Hyung Jun; Kim, Hyun Jo; Jang, Won Ho; Kim, Dong Won

doi:2007.62.6.554

Tuberc Respir Dis > Volume 62(6); 2007 > Article

Review Article

Tuberculosis and Respiratory Diseases 2007;62(6):554-559.
DOI: https://doi.org/10.4046/trd.2007.62.6.554 Published online June 1, 2007.

A Case of Lymphangioleiomyomatosis Combined with Chylothorax and Bilateral Pneumothoraces.

Jong Hwa Kim, Yang Ki Kim, Jung Hyun Kim, Young Mok Lee, Ki Up Kim, Soo Taek Uh, Hyung Jun Noh, Hyun Jo Kim, Won Ho Jang, Dong Won Kim

¹Division of Respiratory & Allergy Medicine, Department of Internal Medicine, Soonchunhyang University, School of Medicine, Seoul, Korea. uhs@hosp.sch.ac.kr
²Department of Radiology, Soonchunhyang University, School of Medicine, Seoul, Korea.
³Department of Chest Surgery, Soonchunhyang University, School of Medicine, Seoul, Korea.
⁴Department of Clinical Pathology, Soonchunhyang University, School of Medicine, Seoul, Korea.

Abstract

A 29-year-old female patient presented with exertional dyspnea that she had suffered with for 2 months. The chest X-ray displayed right pleural effusion that was diagnosed as chyle, according to the level of cholesterol and triglyceride in the pleural fluid. VATS (video assisted thoracic surgery) of the right lung was performed on the 7th day of hospitalization for obtaining a lung biopsy and to control the of pneumochylothorax. On the 11th hospitalization day, VATS of left lung was also performed to control the recurrent pneumothorax via pleurodesis. The lung biopsy showed moderate amounts of spindle-shaped and rounded cells (so-called LAM cells), which were reactive to actin and HMB45 (on immunohistochemical stains). We report here on a rare case of lymphangioleiomyomatosis combined with chylothorax and bilateral pneumothroraces.

Key Words: Lymphangioleiomyomatosis, Chylothorax, Pneumothorax