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Cancer Research and Treatment > Accepted Articles
doi: https://doi.org/10.4143/crt.2021.313    [Accepted]
Clinical Characteristics and Treatment Outcomes of Childhood Acute Promyelocytic Leukemia in Korea: a Nationwide Multicenter Retrospective Study by Korean Pediatric Oncology Study Group
Kyung Mi Park1, Keon Hee Yoo2, Seong Koo Kim3, Jae Wook Lee3, Nack-Gyun Chung3, Hee Young Ju2, Hong Hoe Koo2, Chul Joo Lyu4, Seung Min Han4, Jung Woo Han4, Jung Yoon Choi5, Kyung Taek Hong5, Hyoung Jin Kang5, Hee Young Shin5, Ho Joon Im6, Kyung-Nam Koh6, Hyery Kim6, Hoon Kook7, Hee Jo Baek7, Bo Ram Kim7, Eu Jeen Yang8, Jae Young Lim9, Eun Sil Park9, Eun Jin Choi10, Sang Kyu Park11, Jae Min Lee12, Ye Jee Shim13, Ji Yoon Kim14, Ji Kyoung Park15, Seom Kim Kong16, Young Bae Choi17, Bin Cho3, Young Tak Lim8
1Department of Pediatrics, Dongnam Institute of Radiological and Medical Sciences, Busan, Korea
2Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
3Department of Pediatrics, Seoul St.Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
4Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
5Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Cancer Institute, Seoul, Korea
6Division of Pediatric Hematology and Oncology, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine Seoul, Korea
7Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University medical school, Gwangju, Korea
8Department of Pediatrics, Pusan National University School of Medicines, Yangsan, Korea
9Department of Pediatrics, Gyeongsang National University College of Medicine, Jinju, Korea
10Department of Pediatrics, School of Medicine, Catholic University of Daegu, Daegu, Korea
11Department of Pediatrics, School of Medicine, University of Ulsan, Ulsan, Korea
12Department of Pediatrics, College of Medicine, Yeungnam University, Daegu, Korea
13Department of Pediatrics, Keimyung University School of Medicine, Keimyung University Dongsan Hospital, Daegu, Korea
14Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea
15Department of Pediatrics, Inje University College of Medicine, Busan Paik Hospital, Busan, Korea
16Department of Pediatrics, Kosin University of Medicine, Busan, Korea
17Departments of Pediatrics, Ajou University School of Medicine, Suwon, Korea
Correspondence  Bin Cho ,Tel: 82-2-2258-6187, Fax: 82-2-537-4544, Email: chobinkr@catholic.ac.kr
Young Tak Lim ,Tel: 82-55-360-3165, Fax: 82-55-360-2181 , Email: limyt@pusan.ac.kr
Received: March 9, 2021;  Accepted: April 17, 2021.  Published online: April 20, 2021.
*Kyung Mi Park and Keon Hee Yoo contributed equally to this work.
ABSTRACT
Purpose
Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea.
Materials and Methods
Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively.
Results
Of 801 acute myeloid leukemia(AML) children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3-18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had WBC count greater than 10x109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid (ATRA) and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 (86.8%) patients achieved complete remission (CR) after induction chemotherapy. The causes of death were 3 intracranial hemorrhage (ICH), 1 cerebral infarction, and 1 sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival rate (EFS) and overall survival rates (OS) were 82.1 ± 4.4%, 89.7 ± 5.1% respectively. The 4-yr OS was significantly higher in patients with initial WBC <10x109/L than in those with initial WBC ≥10x109/L (p=0.02).
Conclusion
This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.
Key words: APL, ATRA, Childhood
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