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Cancer Res Treat. 2022 Jan;54(1):269-276. doi: 10.4143/crt.2021.313. Epub 2021 Apr 20.

Clinical Characteristics and Treatment Outcomes of Childhood Acute Promyelocytic Leukemia in Korea: A Nationwide Multicenter Retrospective Study by Korean Pediatric Oncology Study Group.

Cancer research and treatment

Kyung Mi Park, Keon Hee Yoo, Seong Koo Kim, Jae Wook Lee, Nack-Gyun Chung, Hee Young Ju, Hong Hoe Koo, Chuhl Joo Lyu, Seung Min Han, Jung Woo Han, Jung Yoon Choi, Kyung Taek Hong, Hyoung Jin Kang, Hee Young Shin, Ho Joon Im, Kyung-Nam Koh, Hyery Kim, Hoon Kook, Hee Jo Baek, Bo Ram Kim, Eu Jeen Yang, Jae Young Lim, Eun Sil Park, Eun Jin Choi, Sang Kyu Park, Jae Min Lee, Ye Jee Shim, Ji Yoon Kim, Ji Kyoung Park, Seom Gim Kong, Young Bae Choi, Bin Cho, Young Tak Lim

Affiliations

  1. Department of Pediatrics, Dongnam Institute of Radiological and Medical Sciences, Busan, Korea.
  2. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  3. Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
  4. Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
  5. Department of Pediatrics, Seoul National University Cancer Institute, Seoul National University College of Medicine, Seoul, Korea.
  6. Division of Pediatric Hematology and Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
  7. Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University medical school, Gwangju, Korea.
  8. Department of Pediatrics, Pusan National University School of Medicines, Yangsan, Korea.
  9. Department of Pediatrics, Gyeongsang National University College of Medicine, Jinju, Korea.
  10. Department of Pediatrics, Daegu Catholic University School of Medicine, Daegu, Korea.
  11. Department of Pediatrics, University of Ulsan School of Medicine, Ulsan, Korea.
  12. Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea.
  13. Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Korea.
  14. Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea.
  15. Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.
  16. Department of Pediatrics, Kosin University of Medicine, Busan, Korea.
  17. Departments of Pediatrics, Ajou University School of Medicine, Suwon, Korea.

PMID: 33887821 DOI: 10.4143/crt.2021.313

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 children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020).

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.

Keywords: Acute promyelocytic leukemia; All-trans retinoic acid; Childhood

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