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Epidemiology and clinical long-term outcome of childhood aplastic anemia in korea for 15 years: Retrospective study of the Korean Society of Pediatric Hematology Oncology (KSPHO)

Title
Epidemiology and clinical long-term outcome of childhood aplastic anemia in korea for 15 years: Retrospective study of the Korean Society of Pediatric Hematology Oncology (KSPHO)
Authors
Jeong D.C.Chung N.G.Kang H.J.Koo H.H.Kook H.Kim S.K.Kim S.Y.Kim H.S.Kim H.M.Park K.D.Park S.K.Park J.S.Park J.E.Park H.J.Park Y.S.Seo J.J.Sung K.W.Shin H.Y.Ahn H.S.Ryu K.H.Yoo E.S.Lyu C.J.Lee K.S.Lee K.C.Lee S.Y.Lee Y.H.Lim Y.T.Lim Y.J.Jung H.L.Cho B.Choi Y.M.Hah J.O.Hwang T.J.Kim H.K.
Ewha Authors
유경하유은선
SCOPUS Author ID
유경하scopus; 유은선scopus
Issue Date
2011
Journal Title
Journal of Pediatric Hematology/Oncology
ISSN
1077-4114JCR Link
Citation
vol. 33, no. 3, pp. 172 - 178
Indexed
SCI; SCIE; SCOPUS WOS scopus
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. © 2011 Lippincott Williams & Wilkins, Inc.
DOI
10.1097/MPH.0b013e31820826a8
Appears in Collections:
의과대학 > 의학과 > Journal papers
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