Clinical Features and Treatment Outcomes of Langerhans Cell Histiocytosis: A Nationwide Survey From Korea Histiocytosis Working Party

Title
Clinical Features and Treatment Outcomes of Langerhans Cell Histiocytosis: A Nationwide Survey From Korea Histiocytosis Working Party
Author(s)
이재민김보은[김보은]고경남[고경남]서진경[서진경]임호준[임호준]송준섭[송준섭]이지원[이지원]강형진[강형진]박경덕[박경덕]신희영[신희영]최형수[최형수]이수현[이수현]유건희[유건희]성기웅[성기웅]구홍회[구홍회]정혜림[정혜림]정낙균[정낙균]조빈[조빈]김학기[김학기]유철주[유철주]백희조[백희조]국훈[국훈]박준은[박준은]박현진[박현진]박병규[박병규]유은선[유은선]유경하[유경하]이건수[이건수]김흥식[김흥식]박은실[박은실]윤회수[윤회수]이광철[이광철]이미정[이미정]임영탁[임영탁]김황민[김황민]박상규[박상규]박정아[박정아]김순기[김순기]박미림[박미림]임연정[임연정]이영호[이영호]서종진[서종진]
Keywords
DIABETES-INSIPIDUS; CHILDREN; THERAPY; SOCIETY; DISEASE; RISK
Issue Date
201403
Publisher
LIPPINCOTT WILLIAMS & WILKINS
Citation
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, v.36, no.2, pp.125 - 133
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.
URI
http://hdl.handle.net/YU.REPOSITORY/32817http://dx.doi.org/10.1097/MPH.0000000000000054
ISSN
1077-4114
Appears in Collections:
의과대학 > 소아청소년과학교실 > Articles
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