2 207

Cited 26 times in

Clinical features and treatment outcomes of langerhans cell histiocytosis: A nationwide survey from Korea histiocytosis working party

 Bo Eun Kim  ;  Kyung-Nam Koh  ;  Jin Kyung Suh  ;  Ho Joon Im  ;  Joon Sup Song  ;  Ji Won Lee  ;  Hyoung Jin Kang  ;  Kyung Duck Park  ;  Hee Young Shin  ;  Hyoung Soo Choi  ;  Soo Hyun Lee  ;  Keon Hee Yoo  ;  Ki Woong Sung  ;  Hong Hoe Koo  ;  Hye Lim Jung  ;  Nak-Gyun Chung  ;  Bin Cho  ;  Hack Ki Kim  ;  Chuhl Joo Lyu  ;  Hee Jo Baek  ;  Hoon Kook  ;  Jun Eun Park  ;  Hyeon Jin Park  ;  Byung-Kiu Park  ;  w Eun Sun Yoo  ;  Kyung Ha Ryu  ;  Kun Soo Lee  ;  Heung Sik Kim  ;  Jae Min Lee  ;  Eun Sil Park  ;  Hoi Soo Yoon  ;  Kwang Chul Lee  ;  ww Mee Jeong Lee  ;  Young Tak Lim  ;  Hwang Min Kim  ;  Sang Kyu Park  ;  Jeong-A Park  ;  Soon Ki Kim  ;  Meerim Park  ;  Yeon-Jung Lim  ;  Young Ho Lee  ;  Jong Jin Seo 
 Journal of Pediatric Hematology Oncology, Vol.36(2) : 125-133, 2014 
Journal Title
 Journal of Pediatric Hematology Oncology 
Issue Date
Adolescent ; Child ; Child, Preschool ; Data Collection ; Democratic People's Republic of Korea/epidemiology ; Female ; Histiocytosis/mortality* ; Histiocytosis/pathology* ; Histiocytosis/therapy ; Humans ; Infant ; Infant, Newborn ; Kaplan-Meier Estimate ; Male ; Proportional Hazards Models ; Treatment Outcome ; Young Adult
Langerhans cell histiocytosis ; outcome ; reactivation ; permanent consequence
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.
Full Text
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Lyu, Chuhl Joo(유철주) ORCID logo https://orcid.org/0000-0001-7124-7818
사서에게 알리기


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.