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)

Dae Chul Jeong, Nack Gyun Chung, Hyoung Jin Kang, Hong Hoe Koo, Hoon Kook, Soon Ki Kim, Sun Young Kim, Heung Sik Kim, Hwang Min Kim, Kyung Duk Park, Sang Kyu Park, Jae Sun Park, Jun Eun Park, Hyeon Jin Park, Young Shil Park, Jong Jin Seo, Ki Woong Sung, Hee Young Shin, Hyo Seop Ahn, Kun Hee RyuKyung Ha Ryu, Eun Sun Yoo, Chuhl Joo Lyu, Kun Soo Lee, Kwang Chul Lee, Soon Yong Lee, Young Ho Lee, Young Tak Lim, Yeon Jung Lim, Hye Lim Jung, Bin Cho, Yong Mook Choi, Jeong Ok Hah, Tai Ju Hwang, Hack Ki Kim

Research output: Contribution to journalArticle

19 Citations (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.

Original languageEnglish
Pages (from-to)172-178
Number of pages7
JournalJournal of Pediatric Hematology/Oncology
Volume33
Issue number3
DOIs
Publication statusPublished - 2011 Apr

Keywords

  • aplastic anemia
  • children
  • long-term outcome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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    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., ... Kim, H. K. (2011). 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). Journal of Pediatric Hematology/Oncology, 33(3), 172-178. https://doi.org/10.1097/MPH.0b013e31820826a8