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10개월 영아에서 발병한 단일 폐병변 랑게르한스세포 조직구증의 증례

Other Titles
 Isolated pulmonary Langerhans cell histiocytosis in a 10-month-old infant 
Authors
 Hyejin Jang  ;  Yoon Hee Kim  ;  Kyung Won Kim  ;  Myung Hyun Sohn  ;  Chuhl Joo Lyu 
Citation
 Allergy Asthma & Respiratory Disease, Vol.6(3) : 179-183, 2018 
Journal Title
 Allergy Asthma & Respiratory Disease 
ISSN
 2288-0402 
Issue Date
2018
Keywords
Langerhans cell histiocytosis ; Lung biopsy ; Chemotherapy ; Infant
Abstract
Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation and accumulation of abnormal dendritic (Langerhans) cells in various organs. Pulmonary involvement, although rare in children, has been reported in 20%–50% of childhood cases of multisystem LCH. Isolated pulmonary LCH in children, especially in infants, is still rarer, but should be suspected in those with cystic lung disease. We report a case of a 10-month-old boy who presented with chronic dyspnea and whose chest computed tomography (CT) scan demonstrated cystic lesions. Lung biopsy established the diagnosis of LCH; microscopy revealed a background of lymphocytes and eosinophils with kidney-shaped abnormal cells. These abnormal cells were positive for S-100, CD207 (Langerin), and CD1a on immunohistochemical staining. Chemotherapy was administered using a cytotoxic agent (vinblastine) and a steroid. After 12 weeks of induction chemotherapy, although no significant change in cyst size was noted on chest CT, clinical symptoms improved. Consolidation chemotherapy was then administered for 1 year. Thereafter, chest CT findings demonstrated a significant decrease in cyst size and a significant increase in the volume of normal lung parenchyma. Therefore, aggressive treatment of isolated pulmonary LCH in infants with severe tissue destruction and symptoms seems warranted. (Allergy Asthma Respir Dis 2018;6:179-183)
Files in This Item:
T201804214.pdf Download
DOI
10.4168/aard.2018.6.3.179
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
김경원(Kim, Kyung Won) ORCID logo https://orcid.org/0000-0003-4529-6135
김윤희(Kim, Yoon Hee) ORCID logo https://orcid.org/0000-0002-2149-8501
손명현(Sohn, Myung Hyun) ORCID logo https://orcid.org/0000-0002-2478-487X
유철주(Lyu, Chuhl Joo) ORCID logo https://orcid.org/0000-0001-7124-7818
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/165573
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