Yang Eu Jeen, Park Kyung Mi, Lee Jae Min, Hah Jeong Ok, Park Sang Kyu, Suh Jin Kyung, Kim Ji Yoon, Lee Kun Soo, Park Jikyoung, Park Eun Sil, Lim Jaeyoung, Shim Ye Jee, Kim Heung Sik, Kong Seom Gim, Chueh Heewon, Choi Eun Jin, Park Jeong A, Lim Young Tak
a Department of Pediatrics , Pusan National University Children's Hospital, Pusan National University School of Medicine , Yangsan , Republic of Korea.
b Department of Pediatrics , College of Medicine, Yeungnam University , Daegu , Republic of Korea.
Pediatr Hematol Oncol. 2018 May;35(4):276-287. doi: 10.1080/08880018.2018.1483986. Epub 2019 Jan 11.
We aimed to evaluate treatment outcomes of pediatric acute lymphoblastic leukemia (ALL) subgroups by risk-stratification, in the Yeungnam region of Korea.
We reviewed the courses of 409 newly diagnosed ALL patients from January 2004 to December 2013 in the Yeungnam region.
All patients were classified into three risk groups: standard risk (SR, n=212), high risk (HR, n=153) and very high risk (VHR, n=44). The mean follow-up time was 73.6 ± 39.4 months. The 7-year event-free survival (EFS) and overall survival (OS) rates were 78.7 ± 2.1% and 86.8 ± 1.8%, respectively. Significant 7-year EFS and OS rates for SR (84.0 ± 2.7%, 93.7 ± 1.8%), HR (76.5 ± 3.5%, 82.1 ± 3.3%), and VHR (60.6 ± 7.5%, 69.9 ± 7.5%) were observed (P<0.001), respectively. Relapse occurred in 52 patients, and the cumulative 7-year incidence of relapse differed according to risk groups (SR vs. HR vs. VHR=12.6% vs. 14.0% vs. 29.6%, P=0.003).For the 46 relapsed patients who were treated, the 3-year EFS and OS were 42.3 ± 8.3%and 46.4± 8.4%. Among the 44 VHR patients, EFS was not significantly different between the chemotherapy-treated patients and those received hematopoietic stem cell transplantation (P=0.533). The 7-year EFS of the hyperleukocytosis subgroup (24 cases, 14 under 10 years of age)showed a tendency for better prognosis than that of the other VHR subgroups (P=0.178).
Our results revealed improved outcomes in pediatric ALL patients with risk-stratified therapy. The hyperleukocytosis subgroup without any combined chromosomal abnormalities may respond favorably to chemotherapy alone after first complete remission.
我们旨在评估韩国岭南地区按风险分层的小儿急性淋巴细胞白血病（ALL）亚组的治疗结果。
我们回顾了2004年1月至2013年12月在岭南地区新诊断的409例ALL患者的病程。
所有患者被分为三个风险组：标准风险（SR，n = 212）、高风险（HR，n = 153）和极高风险（VHR，n = 44）。平均随访时间为73.6±39.4个月。7年无事件生存率（EFS）和总生存率（OS）分别为78.7±2.1%和86.8±1.8%。观察到SR组（84.0±2.7%，93.7±1.8%）、HR组（76.5±3.5%，82.1±3.3%）和VHR组（60.6±7.5%，69.9±7.5%）的7年EFS和OS率有显著差异（P<0.001）。52例患者发生复发，累积7年复发率因风险组而异（SR组vs. HR组vs. VHR组=12.6% vs. 14.0% vs. 29.6%，P = 0.003）。对于接受治疗的46例复发患者，3年EFS和OS分别为42.3±8.3%和46.4±8.4%。在44例VHR患者中，化疗治疗患者与接受造血干细胞移植的患者之间的EFS无显著差异（P = 0.533）。高白细胞血症亚组（24例，14例年龄在10岁以下）的7年EFS显示出比其他VHR亚组预后更好的趋势（P = 0.178）。
我们的结果显示风险分层治疗的小儿ALL患者预后有所改善。首次完全缓解后，无任何合并染色体异常的高白细胞血症亚组可能对单纯化疗反应良好。
