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)
Author(s)
하정옥강형진[강형진]정대철[정대철]구홍회[구홍회]김학기[김학기]서종진[서종진]유경하[유경하]김흥식[김흥식]국훈[국훈]정혜림[정혜림]
Keywords
BONE-MARROW-TRANSPLANTATION; STEM-CELL TRANSPLANTATION; COLONY-STIMULATING FACTOR; CORD BLOOD TRANSPLANTATION; SAA WORKING PARTY; IMMUNOSUPPRESSIVE THERAPY; ANTITHYMOCYTE GLOBULIN; FOLLOW-UP; ANTILYMPHOCYTE GLOBULIN; EUROPEAN GROUP
Issue Date
201104
Publisher
LIPPINCOTT WILLIAMS & WILKINS
Citation
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, v.33, no.3, pp.172 - 178
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.
URI
http://hdl.handle.net/YU.REPOSITORY/25352http://dx.doi.org/10.1097/MPH.0b013e31820826a8
ISSN
1077-4114
Appears in Collections:
의과대학 > 소아청소년과학교실 > Articles
Files in This Item:
There are no files associated with this item.
Export
RIS (EndNote)
XLS (Excel)
XML


qrcode

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

BROWSE