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epidemiology of Clinical Long-term outcome of chilchood Aplastic anemia in Korea for 15 years: Retrospective study of Korean Society of Pediatric Hematology Oncology(KSPHO)

Authors
 Jeong, Dae Chul  ;  Chung, Nack Gyun  ;  Kang, Hyoung Jin  ;  Koo, Hong Hoe  ;  Kook, Hoon  ;  Kim, Soon Ki  ;  Kim, Sun Young  ;  Kim, Heung Sik  ;  Kim, Hwang Min  ;  Park, Kyung Duk  ;  Park, Sang Kyu  ;  Park, Jae Sun  ;  Park, Jun Eun  ;  Park, Hyeon Jin  ;  Park, Young Shil  ;  Seo, Jong Jin  ;  Sung, Ki Woong  ;  Shin, Hee Young  ;  Ahn, Hyo Seop  ;  Ryu, Kun Hee  ;  Ryu, Kyung Ha  ;  Yoo, Eun Sun  ;  Lyu, Chuhl Joo  ;  Lee, Kun Soo  ;  Lee, Kwang Chul  ;  Lee, Soon Yong  ;  Lee, Young Ho  ;  Lim, Young Tak  ;  Lim, Yeon Jung  ;  Jung, Hye Lim  ;  Cho, Bin  ;  Choi, Yong Mook  ;  Hah, Jeong Ok  ;  Hwang, Tai Ju  ;  Kim, Hack Ki 
Citation
 JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, Vol.33(3) : 172-178, 2011 
Journal Title
 JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY 
ISSN
 1077-4114 
Issue Date
2011
MeSH
Adolescent ; Adult ; Anemia, Aplastic/epidemiology* ; Anemia, Aplastic/etiology ; Anemia, Aplastic/mortality ; Anemia, Aplastic/therapy ; Child ; Child, Preschool ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunosuppressive Agents/therapeutic use ; Incidence ; Infant ; Korea/epidemiology ; Male ; Retrospective Studies ; Treatment Outcome
Keywords
aplastic anemia ; long-term outcome ; children
Abstract
PURPOSE: Aplastic anemia (AA) is a rare hematologic disease characterized by pancytopenia and hypocellular marrow. The Korean Society of Pediatric Hematology Oncology investigated retrospectively the incidence, survival, and transfusion independency according to treatment strategies in AA. METHODS: All the questionnaires were sent to members for medical records. We collected and analyzed 702 available data. RESULTS: The male and female ratio was 1.2, and the median age at diagnosis was 9.3 years. The annual incidence of Korean children with AA was 5.16 per million per year. Constitutional anemia was diagnosed in 44 children. In acquired AA, causes were identified in 39 children. Severe AA (SAA) at initial diagnosis was more common than nonsevere AA. The overall survival was 47.8% with supportive care, 68.1% with immunosuppressive therapy (IST), and 81.8% with hematopoietic stem cell transplantation. In IST, response rate was 65.7%, and relapse rate after response was 54.4% within a median of 23.0 months. The factors with overall survival were severity of disease in supportive care, severity and response in IST, donor type, graft failure, and posttransplant events in hematopoietic stem cell transplantation. CONCLUSIONS: Long-term outcome in AA was dependent on treatment strategies. These Korean results may help research and prospective international clinical trials for childhood AA.
Full Text
http://ovidsp.ovid.com/ovidweb.cgi?T=JS&CSC=Y&NEWS=N&PAGE=fulltext&AN=00043426-201104000-00002&LSLINK=80&D=ovft
DOI
10.1097/MPH.0b013e31820826a8
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
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/95450
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