Tuberc Respir Dis TRD Tuberculosis and Respiratory Diseases 1738-3536 2005-6184 The Korean Academy of Tuberculosis and Respiratory Diseases 10.4046/trd.2007.62.6.554 Case Report A Case of Lymphangioleiomyomatosis Combined with Chylothorax and Bilateral Pneumothoraces Kim Jong-Hwa M.D. 1 Kim Yang-Ki M.D. 1 Kim Jung-Hyun M.D. 1 Lee Young Mok M.D. 1 Kim Ki-Up M.D. 1 Uh Soo-taek M.D. 1 Noh Hyung-Jun M.D. 2 Kim Hyun Jo M.D. 3 Jang Won Ho M.D. 3 Kim Dong-Won M.D. 4 Division of Respiratory & Allergy Medicine, Department of Internal Medicine, Soonchunhyang University, School of Medicine, Seoul, Korea. Department of Radiology, Soonchunhyang University, School of Medicine, Seoul, Korea. Department of Chest Surgery, Soonchunhyang University, School of Medicine, Seoul, Korea. Department of Clinical Pathology, Soonchunhyang University, School of Medicine, Seoul, Korea. Address for correspondence: Soo-taek Uh, M.D. Division of Respiratory & Allergy Medicine, Department of Internal Medicine, Soonchunhyang University School of Medicine, 657, Hannam-dong, Yongsan-gu, Seoul 140-743, Korea. Phone: 82-2-709-9482, Fax: 82-2-709-9554, uhs@hosp.sch.ac.kr 06 2007 30 06 2007 62 6 554 559 28 12 2006 28 05 2007 Copyright © 2007 The Korean Academy of Tuberculosis and Respiratory Diseases 2007

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.

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Pleural fluid before (left) and after (right) centrifugation. The centrifugation resulted in a change of color of the pleural fluid from deep brown (left) to yellow (right) - instead of clearing of pleural fluid- suggesting that the pleural fluid is not empyema. The change of color of pleural fluid may be explained by the precipitation of red cells after centrifugation.

The chest radiograph obtained at 1st hospitalization day displays total haziness of right whole lung field and mild mediastinal deviation to the left hemithorax, suggesting large amount of pleural effusion (A). The chest radiograph obtained on the 3rd hospitalization day displays right pneumothorax. The amount of pleural effusion was considerably decreased after catheter drainage (B). The chest radiograph obtained on the 5th hospitalization day displays bilateral pneumothoraces. The right pleural effusion is nearly resolved (C). The chest CT scan with lung window setting shows multiple, uniform and variable-sized, small cysts with thin and regular wall in both lungs (D,E).

H&E stain (A, B). The cystic space is empty and shows moderate amounts of spindle-shaped and rounded cells (so-called LAM cells, arrow) in its periphery (×40, A). The proliferating LAM cells are spindle, round, or oval shaped (×400, B). Immunohistochemical stain (C,D). LAM cells are positive to smooth muscle actin (×400, C), as well as to HMB45 (×400, D).