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 YumHong Lyeol Lee, Yong Bum Park

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)98-103
Number of pages6
JournalTuberculosis and Respiratory Diseases
Volume66
Issue number2
DOIs
Publication statusPublished - 2009 Feb 1

Fingerprint

Langerhans Cell Histiocytosis
Korea
Lung
Respiratory Function Tests
Langerhans Cells
Pneumothorax
Granuloma
Sputum
Chest Pain
Dyspnea
Biopsy

Keywords

  • Histiocytosis
  • Langerhans
  • Langerhans-cell histiocytosis
  • Pulmonary diseases

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Infectious Diseases

Cite this

Clinical features of pulmonary langerhans cell histiocytosis in Korea. / Kim, Chul; Jeong, Sung Hwan; Shim, Jae Jeong; Cha, Seung Ick; Son, Choonhee; Chung, Man Pyo; Park, Hye Yoon; Kim, Young Whan; Park, Jong Sun; Uh, Soo Taek; Park, Choon Sik; Kim, Dong Soon; Cho, Kyung Wook; Song, Jin Woo; Jegal, Yang Jin; Park, Moo Suk; Park, Byung Hoon; Lee, Jin Hwa; Hur, Jin Won; Yum, Ho Kee; Lee, Hong Lyeol; Park, Yong Bum.

In: Tuberculosis and Respiratory Diseases, Vol. 66, No. 2, 01.02.2009, p. 98-103.

Research output: Contribution to journalArticle

Kim, C, Jeong, SH, Shim, JJ, Cha, SI, Son, C, Chung, MP, Park, HY, Kim, YW, Park, JS, Uh, ST, Park, CS, Kim, DS, Cho, KW, Song, JW, Jegal, YJ, Park, MS, Park, BH, Lee, JH, Hur, JW, Yum, HK, Lee, HL & Park, YB 2009, 'Clinical features of pulmonary langerhans cell histiocytosis in Korea', Tuberculosis and Respiratory Diseases, vol. 66, no. 2, pp. 98-103. https://doi.org/10.4046/trd.2009.66.2.98
Kim, Chul ; Jeong, Sung Hwan ; Shim, Jae Jeong ; Cha, Seung Ick ; Son, Choonhee ; Chung, Man Pyo ; Park, Hye Yoon ; Kim, Young Whan ; Park, Jong Sun ; Uh, Soo Taek ; Park, Choon Sik ; Kim, Dong Soon ; Cho, Kyung Wook ; Song, Jin Woo ; Jegal, Yang Jin ; Park, Moo Suk ; Park, Byung Hoon ; Lee, Jin Hwa ; Hur, Jin Won ; Yum, Ho Kee ; Lee, Hong Lyeol ; Park, Yong Bum. / Clinical features of pulmonary langerhans cell histiocytosis in Korea. In: Tuberculosis and Respiratory Diseases. 2009 ; Vol. 66, No. 2. pp. 98-103.
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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.",
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AU - Jeong, Sung Hwan

AU - Shim, Jae Jeong

AU - Cha, Seung Ick

AU - Son, Choonhee

AU - Chung, Man Pyo

AU - Park, Hye Yoon

AU - Kim, Young Whan

AU - Park, Jong Sun

AU - Uh, Soo Taek

AU - Park, Choon Sik

AU - Kim, Dong Soon

AU - Cho, Kyung Wook

AU - Song, Jin Woo

AU - Jegal, Yang Jin

AU - Park, Moo Suk

AU - Park, Byung Hoon

AU - Lee, Jin Hwa

AU - Hur, Jin Won

AU - Yum, Ho Kee

AU - Lee, Hong Lyeol

AU - Park, Yong Bum

PY - 2009/2/1

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N2 - 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.

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