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埃及儿童癌症医院的急性早幼粒细胞白血病

Acute promyelocytic leukemia in children cancer hospital Egypt.

作者信息

Semary Samah, Hammad Mahmoud, Yassin Dina, El Sharkawy Nahla, Soliman Sonya, Salem Sherine, Ezzat Emad, Mosa Ahmed, Ahmed Sonia

机构信息

Department of Clinical Oncology, Faculty of Medicine, Beni-Suef University, Beni-Suef, Egypt.

Department of Pediatric Oncology, Children's Cancer Hospital Egypt (CCHE-57357), Cairo, Egypt.

出版信息

Discov Oncol. 2024 Jun 11;15(1):223. doi: 10.1007/s12672-024-01037-6.

Abstract

BACKGROUND

Pediatric acute promyelocytic leukemia (APL) accounts for 5 to 15% of all myelocytic leukemia. A retrospective analysis of pediatric patients diagnosed and treated with APL was conducted at CCHE from July 2012 to the end of December 2019, to report the prevalence, clinical characteristics, results, and risk factors associated with induction failure and early death.

RESULT

Sixty-two patients were reported, with an age greater than ten, an initial poor coagulation profile, and a total leukocyte count (TLC) greater than 30 10/mm influencing 5-year overall (OS) and event-free survival (EFS), as well as a high promyelocyte count affecting 5-year EFS. Patients received a regimen based on the COG AAML0631 protocol. High-risk patients with an initial TLC > 10 × 10/mm and an initial promyelocytic count of 30% or more with a substantial P-value are prognostic markers for early death during induction. In females, wild FLT3 increases the risk of differentiation syndrome (DS). Receiving steroids with all-trans retinoic acid (ATRA) induction may reduce the occurrence of DS. Relapse alters the outcome. In the current study, 45 patients are alive in complete remission, with a 5-year OS of 72.5% and a 5-year EFS of 69.4%, respectively.

CONCLUSION

Pediatric APL outcomes are influenced by age above 10, an initial poor coagulation profile, and a promyelocyte count of more than 10%. An initial leukocyte count of more than 10 × 10/mm and an initial promyelocytic count of more than 30% increase the risk of early death. Receiving steroids with ATRA may reduce the occurrence of DS.

摘要

背景

儿童急性早幼粒细胞白血病(APL)占所有髓细胞白血病的5%至15%。2012年7月至2019年12月底在开罗儿童癌症医院(CCHE)对诊断并接受治疗的儿童APL患者进行了回顾性分析,以报告其患病率、临床特征、治疗结果以及与诱导失败和早期死亡相关的危险因素。

结果

共报告62例患者,年龄大于10岁、初始凝血功能差以及总白细胞计数(TLC)大于30×10⁹/mm影响5年总生存(OS)和无事件生存(EFS),而早幼粒细胞计数高影响5年EFS。患者接受基于儿童肿瘤协作组(COG)AAML0631方案的治疗方案。初始TLC>10×10⁹/mm且初始早幼粒细胞计数为30%或更高且P值显著的高危患者是诱导期早期死亡的预后标志物。在女性中,野生型FLT3增加分化综合征(DS)的风险。接受类固醇联合全反式维甲酸(ATRA)诱导治疗可能会降低DS的发生率。复发会改变治疗结果。在本研究中,45例患者处于完全缓解状态存活,5年OS率和5年EFS率分别为72.5%和69.4%。

结论

10岁以上年龄、初始凝血功能差以及早幼粒细胞计数超过10%会影响儿童APL的治疗结果。初始白细胞计数超过10×10⁹/mm以及初始早幼粒细胞计数超过30%会增加早期死亡风险。接受类固醇联合ATRA治疗可能会降低DS的发生率。

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