Nationwide survey of pediatric hypodiploid acute lymphoblastic leukemia in Japan
Nationwide survey of pediatric hypodiploid acute lymphoblastic leukemia in Japan
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DOI：
10.1111/ped.14006
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发表时间：
2019-11-21
影响因子：
1.4
通讯作者：
Koh, Katsuyoshi
中科院分区：
文献类型：
--
作者：
Ishimaru, Sae;Okamoto, Yasuhiro;Koh, Katsuyoshi
背景倍性是儿童急性淋巴细胞白血病（ALL）的一个非常重要的预后因素。尽管目前进行了强化化疗，亚二倍体 ALL 儿童的预后仍很差。关于日本儿童亚二倍体 ALL 的研究很少。方法我们回顾性收集1997年至2012年日本四个独立临床研究组进行的前瞻性多中心试验登记中亚二倍体ALL病例的临床资料。结果本研究共分析了117例亚二倍体ALL患者。染色体数量为 45 条、44 条和少于 44 条的患者分别为 101 名、8 名和 8 名。 5 年总生存率差异显着：染色体数为 45、44 和少于 44 的患者的 5 年总生存率分别为 86.0%、87.5% 和 62.5%（P = 0.037）。在这 8 名拥有 44 条染色体的患者中，有 7 名存活，其中 5 名患者在未接受造血干细胞移植（HSCT）的情况下保持完全缓解。 8 例染色体少于 44 条的患者中，6 例对泼尼松龙反应良好，无一例诱导失败，但复发率较高（5/8）。没有患者出现中枢神经系统复发。 4 名患者复发后接受 HSCT，但只有 1 名存活。结论 正如其他国家之前报道的那样，染色体少于 44 条的日本 ALL 患者的预后较差。尽管样本量很小，但拥有 44 条染色体的患者的预后比之前报道的要好。需要包括国际合作在内的进一步研究来改善染色体少于 44 条的儿科 ALL 患者的预后。
Background Ploidy is a highly significant prognostic factor for pediatric acute lymphoblastic leukemia (ALL). Children with hypodiploid ALL have poor outcomes despite current intensive chemotherapy. Little has been investigated with regard to hypodiploid ALL in Japanese children. Methods We retrospectively collected clinical data on hypodiploid ALL cases from the registries of prospective multicenter trials conducted by the four independent clinical study groups in Japan between 1997 and 2012. Results A total of 117 ALL patients with hypodiploidy were analyzed in this study. There were 101, eight, and eight patients with 45, 44, and fewer than 44 chromosomes, respectively. The 5 year overall survival rates differed significantly: 86.0%, 87.5%, and 62.5% for patients with 45, 44, and fewer than 44 chromosomes, respectively (P = 0.037). Of the eight patients with 44 chromosomes, seven were alive, including five patients who maintained complete remission without undergoing hematopoietic stem cell transplantation (HSCT). Of the eight patients with fewer than 44 chromosomes, six were good responders to prednisolone and none had induction failure, but the relapse rate was high (5/8). No patients had central nervous system relapse. Four patients underwent HSCT after relapse, but only one survived. Conclusions Outcomes of Japanese ALL patients with fewer than 44 chromosomes were poor, as previously reported in other countries. Although the sample size was small, patients with 44 chromosomes had better prognoses than those previously reported. Further studies including international collaboration are needed to improve outcomes for pediatric ALL patients with fewer than 44 chromosomes.