Tuberc Respir Dis.  2009 Feb;66(2):98-103.

Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea

• 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

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.

Keyword

Langerhans; Langerhans-cell histiocytosis; Pulmonary diseases; Histiocytosis

MeSH Terms

Biopsy
Chest Pain
Cough
Dyspnea
Female
Follow-Up Studies
Granuloma
Histiocytosis
Histiocytosis, Langerhans-Cell
Humans
Incidence
Korea
Langerhans Cells
Lung
Lung Diseases
Male
Pneumothorax
Surveys and Questionnaires
Respiratory Function Tests
Retrospective Studies
Sputum

Figure

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  Figure 1 Age and sex distribution in patients with PLCH.

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  Figure 2 Smoking status before and after diagnosis in patients with PLCH.

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