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KMID : 0939920220540010269
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대한암학회지
2022 Volume.54 No. 1 p.269 ~ p.276
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Clinical Characteristics and Treatment Outcomes of Childhood Acute Promyelocytic Leukemia in Korea: A Nationwide Multicenter Retrospective Study by Korean Pediatric Oncology Study Group
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Park Kyung-Mi
Yoo Keon-Hee
Kim Seong-Koo
Lee Jae-Wook
Chung Nack-Gyun
Ju Hee-Young
Koo Hong-Hoe
Lyu Chuhl-Joo
Han Seung-Min
Han Jung-Woo
Choi Jung-Yoon
Hong Kyung-Taek
Kang Hyoung-Jin
Shin Hee-Young
Im Ho-Joon
Koh Kyung-Nam
Kim Hye-Ry
Kook Hoon
Baek Hee-Jo
Kim Bo-Ram
Yang Eu-Jeen
Lim Jae-Young
Park Eun-Sil
Choi Eun-Jin
Park Sang-Kyu
Lee Jae-Min
Shim Ye-Jee
Kim Ji-Yoon
Park Ji-Kyoung
Kong Seom-Gim
Choi Young-Bae
Cho Bin
Lim Young-Tak
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Abstract
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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.
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KEYWORD
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Acute promyelocytic leukemia, All-trans retinoic acid, Childhood
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