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 Ryu & 15 others Kyung 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

16 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 1

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Aplastic Anemia
Hematology
Korea
Epidemiology
Retrospective Studies
Pediatrics
Immunosuppressive Agents
Hematopoietic Stem Cell Transplantation
Therapeutics
Pancytopenia
Hematologic Diseases
Incidence
Rare Diseases
Medical Records
Anemia
Bone Marrow
Tissue Donors
Clinical Trials
Transplants
Recurrence

Keywords

  • aplastic anemia
  • children
  • long-term outcome

ASJC Scopus subject areas

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

Cite this

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). / 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.

In: Journal of Pediatric Hematology/Oncology, Vol. 33, No. 3, 01.04.2011, p. 172-178.

Research output: Contribution to journalArticle

Jeong, DC, Chung, NG, Kang, HJ, Koo, HH, Kook, H, Kim, SK, Kim, SY, Kim, HS, Kim, HM, Park, KD, Park, SK, Park, JS, Park, JE, Park, HJ, Park, YS, Seo, JJ, Sung, KW, Shin, HY, Ahn, HS, Ryu, KH, Ryu, KH, Yoo, ES, Lyu, CJ, Lee, KS, Lee, KC, Lee, SY, Lee, YH, Lim, YT, Lim, YJ, Jung, HL, Cho, B, Choi, YM, Hah, JO, Hwang, TJ & Kim, HK 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, vol. 33, no. 3, pp. 172-178. https://doi.org/10.1097/MPH.0b013e31820826a8
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. / 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). In: Journal of Pediatric Hematology/Oncology. 2011 ; Vol. 33, No. 3. pp. 172-178.
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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.",
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T1 - Epidemiology and clinical long-term outcome of childhood aplastic anemia in korea for 15 years

T2 - Retrospective study of the Korean Society of Pediatric Hematology Oncology (KSPHO)

AU - Jeong, Dae Chul

AU - Chung, Nack Gyun

AU - Kang, Hyoung Jin

AU - Koo, Hong Hoe

AU - Kook, Hoon

AU - Kim, Soon Ki

AU - Kim, Sun Young

AU - Kim, Heung Sik

AU - Kim, Hwang Min

AU - Park, Kyung Duk

AU - Park, Sang Kyu

AU - Park, Jae Sun

AU - Park, Jun Eun

AU - Park, Hyeon Jin

AU - Park, Young Shil

AU - Seo, Jong Jin

AU - Sung, Ki Woong

AU - Shin, Hee Young

AU - Ahn, Hyo Seop

AU - Ryu, Kun Hee

AU - Ryu, Kyung Ha

AU - Yoo, Eun Sun

AU - Lyu, Chuhl Joo

AU - Lee, Kun Soo

AU - Lee, Kwang Chul

AU - Lee, Soon Yong

AU - Lee, Young Ho

AU - Lim, Young Tak

AU - Lim, Yeon Jung

AU - Jung, Hye Lim

AU - Cho, Bin

AU - Choi, Yong Mook

AU - Hah, Jeong Ok

AU - Hwang, Tai Ju

AU - Kim, Hack Ki

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N2 - 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.

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