Jastaniah Wasil, Alsultan Abdulrahman, Al Daama Saad, Ballourah Walid, Bayoumy Mohammad, Al-Anzi Faisal, Al Shareef Omar, Abrar Mohammed Burhan, Al Sudairy Reem, Al Ghemlas Ibrahim
Department of Pediatrics, Faculty of Medicine, Umm AlQura University, Makkah, Saudi Arabia; Princess Noorah Oncology Center, King Saud Bin Abdulaziz University and King Abdulaziz Medical City, Jeddah, Saudi Arabia.
Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Leuk Res. 2017 Jul;58:48-54. doi: 10.1016/j.leukres.2017.04.004. Epub 2017 Apr 12.
Despite the high incidence of Down syndrome (DS) in Arab countires, the incidence and outcomes of myeloid leukemia of DS (ML-DS) have not been studied. We evaluated 206 pediatric acute myeloid leukemia (AML) patients diagnosed between 2005 and 2012 and identified 31 (15%) ML-DS. The incidence of ML-DS was 48 per 100,000 compared to 0.6 per 100,000 for AML in non-DS children. Thus, patients with DS had 80-fold increased risk of ML-DS compared to AML in non-DS children. The median age at diagnosis was 1.8 years, male/female ratio was 1.2, majority (84%) of patients had FAB-M7 subtype, and the cytogenetic abnormalities were normal karyotype (constitutional trisomy 21) in 48%, additional trisomy in 23%, and other aberrations in 29%. Complete remission, cumulative incidences of relapse (CIR), toxic-death, and 5-year event-free survival (EFS) rates were 96.8%, 19.4%, 13.1%, and 67.7±8.4%; respectively. In the present study, multivariate analysis revealed favorable outcome (5-year EFS 86.7±8.8%) for patients with normal karyotype. The incidence and clinical characteristics of ML-DS in Saudi patients were comparable to other reports. However, there is a need to optimize risk stratification and treatment intensity to reduce CIR and toxic death rates to further improve outcomes of patients with ML-DS.
尽管阿拉伯国家唐氏综合征（DS）的发病率很高，但DS相关髓系白血病（ML-DS）的发病率和转归尚未得到研究。我们评估了2005年至2012年间诊断的206例儿童急性髓系白血病（AML）患者，确定了31例（15%）ML-DS。ML-DS的发病率为每10万人48例，而非DS儿童AML的发病率为每10万人0.6例。因此，与非DS儿童的AML相比，DS患者发生ML-DS的风险增加了80倍。诊断时的中位年龄为1.8岁，男女比例为1.2，大多数患者（84%）为FAB-M7亚型，细胞遗传学异常情况为：48%为正常核型（21号染色体三体），23%为额外三体，29%为其他畸变。完全缓解率、累积复发率（CIR）、毒性死亡率和5年无事件生存率（EFS）分别为96.8%、19.4%、13.1%和67.7±8.4%。在本研究中，多变量分析显示核型正常的患者预后良好（5年EFS为86.7±8.8%）。沙特患者中ML-DS的发病率和临床特征与其他报告相当。然而，有必要优化风险分层和治疗强度，以降低CIR和毒性死亡率，从而进一步改善ML-DS患者的转归。
