Tuberc Respir Dis > Volume 66(2); 2009 > Article
Tuberculosis and Respiratory Diseases 2009;66(2):98-103.
DOI:    Published online February 1, 2009.
Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea.
Chul Kim, Sung Hwan Jeong, Jae Jeong Shim, Seung Ick Cha, Choonhee Son, Man Pyo Chung, Hye Yoon Park, Young Whan Kim, Jong Sun Park, Soo Taek Uh, Choon Sik Park, Dong Soon Kim, Kyung Wook Cho, Jin Woo Song, Yang Jin Jegal, Moo Suk Park, Byung Hoon Park, Jin Hwa Lee, Jin Won Hur, Ho Kee Yum, Hong Lyeol Lee, Yong Bum Park
1Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
2Department of Internal Medicine, Guro Hospital, Korea University Medical Center, Seoul, Korea.
3Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea.
4Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea.
5Department of Internal Medicine, Sungkyunkwan University School of Medicine, Seoul, Korea.
6Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
7Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.
8Department of Internal Medicine, Soonchunhyang University College of Medicine, Bucheon, Korea.
9Department of Internal Medicine, Asan Medical Center, Seoul, Korea.
10Department of Internal Medicine, Ulsan University College of Medicine, Ulsan, Korea.
11Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
12Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea.
13Department of Internal Medicine, College of Medicine, Inje University, Busan, Korea.
14Department of Internal Medicine, College of Medicine, Inha University, Incheon, Korea.
15Department of Internal Medicine, College of Medicine, Hallym University, Seoul, Korea.
Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. METHODS: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. RESULTS: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18~67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1~180 months) and only two patients died during this period. CONCLUSION: This study provides a national survey of the patients with PLCH during a long follow-up period.
Key Words: Langerhans, Langerhans-cell histiocytosis, Pulmonary diseases, Histiocytosis

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