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; 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

doi:10.1097/MPH.0b013e31820826a8

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 journal › Article › peer-review

24 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 language	English
Pages (from-to)	172-178
Number of pages	7
Journal	Journal of Pediatric Hematology/Oncology
Volume	33
Issue number	3
DOIs	https://doi.org/10.1097/MPH.0b013e31820826a8
Publication status	Published - 2011 Apr
Externally published	Yes

Keywords

aplastic anemia
children
long-term outcome

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1097/MPH.0b013e31820826a8

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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

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 et al.
In: Journal of Pediatric Hematology/Oncology, Vol. 33, No. 3, 04.2011, p. 172-178.

Research output: Contribution to journal › Article › peer-review

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

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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)",

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.",

keywords = "aplastic anemia, children, long-term outcome",

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

year = "2011",

month = apr,

doi = "10.1097/MPH.0b013e31820826a8",

language = "English",

volume = "33",

pages = "172--178",

journal = "Journal of Pediatric Hematology/Oncology",

issn = "1077-4114",

publisher = "Lippincott Williams and Wilkins",

number = "3",

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TY - JOUR

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

PY - 2011/4

Y1 - 2011/4

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.

AB - 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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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)

Abstract

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