title: Clinical features and treatment outcomes of Langerhans cell histiocytosis: a nationwide survey from Korea histiocytosis working party.
abstract: A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo). Bone was the most frequently affected organ (79.6%) followed by skin (19.2%). Initially, 419 patients (69.5%) had single-system involvement (SS), 85 (14.1%) with multisystem (MS) disease without risk organ involvement (MS-RO), and 99 (16.4%) multisystem disease with risk organ involvement (MS-RO). The 5-year overall survival (OS) rates in the SS, MS-RO, and MS-RO groups were 99.8%, 98.4%, and 77.0%, respectively (P<0.001), and the 5-year reactivation rates were 17.9%, 33.5%, and 34.3%, respectively (P<0.001). The OS rate was lower in patients with RO involvement (P=0.025) and lack of response to initial treatment (P=0.001). MS involvement (P=0.036) was an independent risk factor for reactivation. Permanent consequences were documented in 99 patients (16.4%). Reactivation of disease, MS involvement, and age at diagnosis ≤ 2 years were associated with higher incidence of permanent consequences. This study emphasized that further efforts are required to improve survival of MS-RO patients and reduce reactivation in younger patients with MS involvement.
authors.affiliation: *Department of Pediatrics, Division of Pediatric Hematology/Oncology, Asan Medical Center, University of Ulsan College of Medicine †Department of Pediatrics, Hallym Universtiy College of Medicine ‡Department of Pediatrics, Seoul National University College of Medicine §Department of Pediatrics, Sungkyunkwan University School of Medicine ∥Department of Pediatrics, The Catholic University of Korea College of Medicine ¶Department of Pediatrics, Yonsei University College of Medicine #Department of Pediatrics, Chonnam National University College of Medicine, Gwangju **Department of Pediatrics, Ajou University College of Medicine, Suwon ††Department of Pediatrics, National Cancer Center, Goyang ‡‡Department of Pediatrics, Ewha Womans University School of Medicine, Seoul §§Department of Pediatrics, Kyungpook National University College of Medicine ∥∥Department of Pediatrics, Keimyung University College of Medicine ¶¶Department of Pediatrics, Yeungnam University College of Medicine, Daegu ##Department of Pediatrics, Gyeongsang National University, Jinju ***Department of Pediatrics, Kyung Hee University College of Medicine †††Department of Pediatrics, Korea University College of Medicine ‡‡‡Department of Pediatrics, Dankook University College of Medicine, Cheonan §§§Department of Pediatrics, Pusan National University College of Medicine, Busan ∥∥∥Department of Pediatrics, Yonsei University Wonju College of Medicine, Wonju ¶¶¶Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan ###Department of Pediatrics, Inje University College of Medicine, Busan ****Department of Pediatrics, Inha University College of Medicine, Incheon ††††Department of Pediatrics, Chungbuk National University College of Medicine, Chungju ‡‡‡‡Department of Pediatrics, Chungnam National University College of Medicine, Daejeon §§§§Department of Pediatrics, Hanyang University College of Medicine,
authors.forename: Bo Eun
authors.initials: BE
authors.lastname: Kim
authors.forename: Kyung-Nam
authors.initials: KN
authors.lastname: Koh
authors.forename: Jin Kyung
authors.initials: JK
authors.lastname: Suh
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authors.initials: HJ
authors.lastname: Im
authors.forename: Joon Sup
authors.initials: JS
authors.lastname: Song
authors.forename: Ji Won
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authors.lastname: Lee
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authors.lastname: Kang
authors.forename: Kyung Duck
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authors.lastname: Park
authors.forename: Hee Young
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authors.lastname: Shin
authors.forename: Hyoung Soo
authors.initials: HS
authors.lastname: Choi
authors.forename: Soo Hyun
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authors.lastname: Lee
authors.forename: Keon Hee
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authors.lastname: Yoo
authors.forename: Ki Woong
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authors.lastname: Sung
authors.forename: Hong Hoe
authors.initials: HH
authors.lastname: Koo
authors.forename: Hye Lim
authors.initials: HL
authors.lastname: Jung
authors.forename: Nak-Gyun
authors.initials: NG
authors.lastname: Chung
authors.forename: Bin
authors.initials: B
authors.lastname: Cho
authors.forename: Hack Ki
authors.initials: HK
authors.lastname: Kim
authors.forename: Chuhl Joo
authors.initials: CJ
authors.lastname: Lyu
authors.forename: Hee Jo
authors.initials: HJ
authors.lastname: Baek
authors.forename: Hoon
authors.initials: H
authors.lastname: Kook
authors.forename: Jun Eun
authors.initials: JE
authors.lastname: Park
authors.forename: Hyeon Jin
authors.initials: HJ
authors.lastname: Park
authors.forename: Byung-Kiu
authors.initials: BK
authors.lastname: Park
authors.forename: Eun Sun
authors.initials: ES
authors.lastname: Yoo
authors.forename: Kyung Ha
authors.initials: KH
authors.lastname: Ryu
authors.forename: Kun Soo
authors.initials: KS
authors.lastname: Lee
authors.forename: Heung Sik
authors.initials: HS
authors.lastname: Kim
authors.forename: Jae Min
authors.initials: JM
authors.lastname: Lee
authors.forename: Eun Sil
authors.initials: ES
authors.lastname: Park
authors.forename: Hoi Soo
authors.initials: HS
authors.lastname: Yoon
authors.forename: Kwang Chul
authors.initials: KC
authors.lastname: Lee
authors.forename: Mee Jeong
authors.initials: MJ
authors.lastname: Lee
authors.forename: Young Tak
authors.initials: YT
authors.lastname: Lim
authors.forename: Hwang Min
authors.initials: HM
authors.lastname: Kim
authors.forename: Sang Kyu
authors.initials: SK
authors.lastname: Park
authors.forename: Jeong-A
authors.initials: JA
authors.lastname: Park
authors.forename: Soon Ki
authors.initials: SK
authors.lastname: Kim
authors.forename: Meerim
authors.initials: M
authors.lastname: Park
authors.forename: Yeon-Jung
authors.initials: YJ
authors.lastname: Lim
authors.forename: Young Ho
authors.initials: YH
authors.lastname: Lee
authors.forename: Jong Jin
authors.initials: JJ
authors.lastname: Seo
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003625:Data Collection; D056911:Democratic People's Republic of Korea / Q000453:epidemiology; D005260:Female; D015614:Histiocytosis / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D016016:Proportional Hazards Models; D016896:Treatment Outcome; D055815:Young Adult