Clinical Pathology Department, National Cancer Institute, Cairo University, P.O Box 11796, Kasr Al Eini street, Fom El Khalig, Cairo, Egypt.
Clinical Pathology Department, National Cancer Institute, Cairo University, P.O Box 11796, Kasr Al Eini street, Fom El Khalig, Cairo, Egypt.
Hematol Oncol Stem Cell Ther. 2021 Jun;14(2):119-125. doi: 10.1016/j.hemonc.2020.07.002. Epub 2020 Jul 24.
Acute promyelocytic leukemia is a peculiar disease with few studies that have investigated the prognostic significance of PML/RARA transcript level at diagnosis.
This retrospective study included all cases diagnosed with acute promyelocytic leukemia over the period from June 2015 to March 2019. The normalized copy number (NCN) was tested by real-time polymerase chain reaction at diagnosis, and at the end of induction regimen.
Our study included 83 de novo APL patients, 53 (63.9%) were adults and 30 (36.1%) were children. The median (range) age of our patients was 28.0 (1.0-70.0) years. The pediatric group had a significantly higher prevalence in males (p = 0.02), higher incidence of disseminated intravascular coagulopathy (p = 0.014), and high-risk groups (p = 0.017). At diagnosis, the median NCN (%) of the entire group at 22.5 was set as the cut off value. There was no significant association between NCN at diagnosis and other prognostic variables except for bone marrow promyelocytes (p = 0.006). High-risk group APL patients as well as those presenting with hemorrhage had an inferior overall survival (OS) (p = 0.007; p < 0.001) respectively. PML-RARA NCN at diagnosis did not have an impact on the OS or increased risk of relapse of our patients (p = 0.434; p = 0.721).
the initial PML/RARA tumor burden is not a prognostic factor for APL. The initial TLC at 10x10/L cut off is the most important predictive for OS. Early detection and close monitoring are required to decrease the high rate of early deaths in developing countries.
急性早幼粒细胞白血病是一种特殊的疾病，目前仅有少数研究探讨了初诊时 PML/RARA 转录本水平的预后意义。
本回顾性研究纳入了 2015 年 6 月至 2019 年 3 月期间所有确诊为急性早幼粒细胞白血病的患者。初诊时和诱导治疗结束时采用实时聚合酶链反应检测标准化拷贝数（NCN）。
本研究纳入了 83 例初发急性早幼粒细胞白血病患者，其中 53 例（63.9%）为成人，30 例（36.1%）为儿童。患者的中位（范围）年龄为 28.0（1.0-70.0）岁。儿科组中男性比例明显更高（p=0.02），弥散性血管内凝血发生率更高（p=0.014），且高危组比例更高（p=0.017）。初诊时，整个组别的中位数 NCN（%）为 22.5%，将其设为截断值。除骨髓早幼粒细胞外，NCN 与其他预后变量之间无显著相关性（p=0.006）。高危组急性早幼粒细胞白血病患者和发生出血的患者总体生存（OS）较差（p=0.007；p<0.001）。初诊时 PML-RARA NCN 对患者的 OS 或复发风险无影响（p=0.434；p=0.721）。
初始 PML/RARA 肿瘤负荷不是急性早幼粒细胞白血病的预后因素。10x10/L 截断值的初始 TLC 是 OS 的最重要预测指标。需要早期发现和密切监测，以降低发展中国家早期死亡率高的问题。
