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

doi:10.4046/trd.2009.66.2.98

Journal List > Tuberc Respir Dis > v.66(2) > 1001396

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Kim, Jeong, Shim, Cha, Son, Chung, Park, Kim, Park, Uh, Park, Kim, Cho, Song, Jegal, Park, Park, Lee, Hur, Yum, Lee, and Park: Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea

Original Article

Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2009; 66(2): 98-103.

Published online: 28 February 2009

DOI: https://doi.org/10.4046/trd.2009.66.2.98

Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea

Chul Kim, M.D.⁷, Sung Hwan Jeong, M.D.¹, Jae Jeong Shim, M.D.², Seung-Ick Cha, M.D.³, Choonhee Son, M.D.⁴, Man Pyo Chung, M.D.⁵, Hye Yoon Park, M.D.⁵, Young Whan Kim, M.D.⁶, Jong Sun Park, M.D.⁶, Soo-Taek Uh, M.D.⁷, Choon Sik Park, M.D.⁸

, Dong Soon Kim, M.D.⁹, Kyung Wook Cho, M.D.⁹, Jin Woo Song, M.D.⁹, Yang Jin Jegal, M.D.¹⁰, Moo Suk Park, M.D.¹¹, Byung Hoon Park, M.D.¹¹, Jin Hwa Lee, M.D.¹²

, Jin-Won Hur, M.D.¹³, Ho-Kee Yum, M.D.¹³, Hong-Lyeol Lee, M.D.¹⁴, Yong Bum Park, M.D.¹⁵

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, Ulsan University College of Medicine, Seoul, Korea.

¹⁰Department of Internal Medicine, Ulsan Medical Center, 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.

Address for correspondence: Soo-Taek Uh, M.D., Ph.D. Department of Internal Medicine, Soonchunhyang University Hospital, 22, Daesakwan-gil, Yongsan-gu, Seoul 140-743, Korea Phone: 82-2-709-9554, Fax: 82-2-709-9554, uhs@hosp.sch.ac.kr

Received 20 January 2009 Accepted 9 February 2009

Abstract

Background

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.

Keywords: Langerhans, Langerhans-cell histiocytosis, Pulmonary diseases, Histiocytosis

Figures and Tables

Figure 1

Age and sex distribution in patients with PLCH.

Figure 2

Smoking status before and after diagnosis in patients with PLCH.

Table 1

Clinical symptoms in 56 patients with PLCH

PLCH: pulmonary Langerhans cell histiocytosis.

Table 2

Pulmonary function tests in 56 patients with PLCH

M±SD denotes mean±standard deviation. PLCH: pulmonary Langerhans cell histiocytosis.

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ORCID iDs

Choon Sik Park
https://orcid.org/http://orcid.org/0000-0003-2977-0255

Jin Hwa Lee
https://orcid.org/http://orcid.org/0000-0003-0843-9862

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