Clinical Analysis of Pediatric Acute Leukemias of Ambiguous Lineage: A Single Institution Retrospective Review.

IF 0.9 4区 医学 Q4 HEMATOLOGY
Chen Xia, Chen Xiaoli, Zhu Xiaofan
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引用次数: 0

Abstract

Acute leukemias of ambiguous lineage (ALAL) is a rare type of acute leukemia, referring to a group of disorders characterized by a combination of myeloid, lymphoid, or more lineages, whose incidence is significantly lower in children than adults. Here, we summarized the clinical features and outcomes of 36 pediatric ALAL patients in past 16 years. The patients diagnosed as ALAL based on the criteria of EGIL scoring system in 1998 (EGIL 1998) and/or the 2016 revisions to the WHO classification (WHO 2016) from January 1, 2005 to December 1, 2021 were included, respectively. During follow-up for a median 22 months, the median leukemia-free survival (LFS) was 18 months (0 to 172 mo) and the median overall survival (OS) was 22 months (1 to 173 mo), with a 5-year LFS rate of 67.3±9.2% and a 5-year OS rate of 66.0±10.7%. Patients who sustained negative minimal residual disease after 2 courses of standardized chemotherapy contributed to better 5-year OS (100% vs. 37.2±22.0%, P =0.028) and LFS (100% vs. 46.7±16.6%, P =0.028).

谱系不明确的儿童急性白血病的临床分析:一项单一机构的回顾性回顾。
不明谱系急性白血病(Acute leukemias of ambiguous lineage, ALAL)是一种罕见的急性白血病类型,指的是一组以髓系、淋巴系或更多谱系结合为特征的疾病,其在儿童中的发病率明显低于成人。在此,我们总结了16年来36例小儿ALAL患者的临床特点和预后。分别纳入2005年1月1日至2021年12月1日根据1998年EGIL评分系统(EGIL 1998)和/或2016年WHO分类修订版(WHO 2016)诊断为ALAL的患者。随访22个月,中位无白血病生存期(LFS)为18个月(0 ~ 172个月),中位总生存期(OS)为22个月(1 ~ 173个月),5年LFS率为67.3±9.2%,5年OS率为66.0±10.7%。2个疗程的标准化化疗后最小残留病变为阴性的患者,其5年OS(100%比37.2±22.0%,P=0.028)和LFS(100%比46.7±16.6%,P=0.028)均较好。
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来源期刊
CiteScore
1.90
自引率
8.30%
发文量
415
审稿时长
2.5 months
期刊介绍: ​Journal of Pediatric Hematology/Oncology (JPHO) reports on major advances in the diagnosis and treatment of cancer and blood diseases in children. The journal publishes original research, commentaries, historical insights, and clinical and laboratory observations.
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