目的:描述巴基斯坦儿童急性早幼粒细胞白血病(APL)的预后变量和病程。
方法:队列研究。研究的地点和持续时间:儿科肿瘤科,联合军事医院,拉瓦尔品第,巴基斯坦,从2012年1月到2022年12月。
方法:1-15岁的患者,纳入临床证实的APL伴早幼粒细胞白血病-维甲酸受体α(PML-RARA).初次入院包括彻底检查,记录人口统计学和临床数据,报告时间,治疗前,和社会经济地位。统计学分析采用SPSS25.0,p<0.05有显著性。
结果:本研究包括50例APL。从中,32(64%)为男性,18(34%)为女性。诊断时的平均年龄为7.02±3.86岁。脸色苍白(96%)和发烧(88%)是常见的表现。平均白细胞计数为28.70±35.39x109/L。治疗方案包括48%的国际儿童联盟(ICC)-APL,和52%的三氧化二砷(ATO)。高危病例为54%。中性粒细胞减少症和分化综合征是常见的诱导并发症。超过一个月的延误增加了引产死亡(6.7%至35%,p=0.011),减少无病生存率(DFS),(76.7%至35%,p=0.001),和总生存率(OS),(80%到45%,p=0.007)。随访40.90±45.19个月后,10年OS和DFS分别为66.0%和60.0%,分别。最好的操作系统和DFS,80%,在ATO治疗的标准风险病例中观察到。
结论:中性粒细胞减少症和出血是儿科APL诱导死亡的主要原因。治疗延迟是一个关键的预后因素。基于ATO的治疗更安全,改进的DFS,和操作系统适用于初级卫生保健设置。
背景:急性早幼粒细胞白血病,化疗,中性粒细胞减少症.
OBJECTIVE: To describe the prognostic variables and course of paediatric acute promyelocytic leukaemia (APL) in Pakistan.
METHODS: Cohort study. Place and Duration of the Study: Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, Pakistan, from January 2012 to December 2022.
METHODS: Patients aged 1-15 years, clinically confirmed APL with promyelocytic leukaemia- retinoic acid receptor alpha (PML-RARA) were enrolled. Initial admission included a thorough examination, recording demographic and clinical data, reporting time, prior treatment, and socioeconomic status. Statistical analysis used SPSS 25.0, with significance at p <0.05.
RESULTS: This study included 50 cases of APL. Out of which, 32 (64%) were males and 18 (34%) were females. The mean age at diagnosis was 7.02 ± 3.86 years. Pallor (96%) and fever (88%) were common presentations. The average white blood cell count was 28.70 ± 35.39 x109/L. Treatment protocols include 48% International Consortium for Childhood (ICC)-APL, and 52% arsenic trioxide (ATO). High-risk cases were 54%. Neutropenic fever and differentiation syndrome were common induction complications. Delays over one month increased induction deaths (6.7 to 35%, p = 0.011), reducing disease-free survival (DFS), (76.7 to 35%, p = 0.001), and overall survival (OS), (80 to 45%, p = 0.007). After 40.90 ± 45.19 months\' follow-up, 10-year OS and DFS were 66.0% and 60.0%, respectively. The best OS and DFS, at 80%, were observed in standard-risk cases treated with ATO.
CONCLUSIONS: Neutropenic fever and bleeding were the primary causes of mortality in paediatric APL induction. Treatment delay was a key prognostic factor. ATO-based therapy offered safer, improved DFS, and OS suitable for primary healthcare settings.
BACKGROUND: Acute promyelocytic leukaemia, Chemotherapy, Neutropenic fever.