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鉴定影响沙特阿拉伯儿童急性髓细胞白血病首次复发后预后的因素:多中心 SAPHOS 研究的结果。

Identifying Prognostic Factors That Influence Outcome of Childhood Acute Myeloid Leukemia in First Relapse in Saudi Arabia: Results of the Multicenter SAPHOS Study.

机构信息

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.

King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.

出版信息

Clin Lymphoma Myeloma Leuk. 2018 Dec;18(12):773-780. doi: 10.1016/j.clml.2018.09.001. Epub 2018 Sep 12.

Abstract

BACKGROUND

The outcome of childhood acute myeloid leukemia (AML) in first relapse (rAML) remains poor. Reported overall survival (OS) rates vary between high-income developed countries and those with fewer resources. The OS of rAML in high-income developing countries (HIDCs) has not been reported.

PATIENTS AND MATERIALS

A multicenter study was performed in an HIDC. The outcome of patients with relapsed non-M3/non-Down syndrome AML was evaluated. Three-year OS was computed using the Kaplan-Meier method, and predictors of OS were analyzed using a Cox proportional hazards model.

RESULTS

A total of 88 patients with non-M3/non-Down syndrome AML diagnosed between January 2005 and December 2012 with a first relapse were identified. Their 3-year OS was 22.6% ± 5.4%. Patients with inv(16) and t(8;21) had an OS of 75.0% ± 21.7% and 36.0% ± 16.1%, respectively. Worse outcomes were associated with "other intermediate" and 11q23 rearrangement AML (OS of 9.4% ± 8.7% and 10.7% ± 9.6%, respectively). Patients experiencing time to relapse (TTR) less than 1 year had shorter OS than those with a longer TTR (14.6% ± 5.4% vs. 41.1% ± 11.5%; P = .006). The outcome of patients after stem cell transplantation (SCT) in second complete remission (CR2) was superior compared with no SCT (50.9% ± 11.2% vs. 7.7% ± 4.6%; P = .001). TTR, risk group, CR2, and SCT in CR2 were the most significant predictors for survival.

CONCLUSIONS

rAML remains a clinical challenge. Genetic variability in outcomes was observed. A majority of patients with inv(16) were successfully salvaged post-relapse, whereas patients with 11q23 rearrangement had a poor prognosis. Only one-third of those with t(8;21) rAML survived. Better access to SCT in HIDCs is needed.

摘要

背景

儿童急性髓系白血病(AML)初次复发(rAML)的预后仍然较差。据报道,高收入发达国家和资源较少国家的总生存率(OS)存在差异。高收入发展中国家(HIDC)rAML 的 OS 尚未有报道。

患者和材料

在 HIDC 中进行了一项多中心研究。评估了复发非 M3/非 Down 综合征 AML 患者的预后。采用 Kaplan-Meier 法计算 3 年 OS,并采用 Cox 比例风险模型分析 OS 的预测因素。

结果

共纳入 88 例 2005 年 1 月至 2012 年 12 月诊断为非 M3/非 Down 综合征 AML 且初次复发的患者。他们的 3 年 OS 为 22.6%±5.4%。inv(16)和 t(8;21)患者的 OS 分别为 75.0%±21.7%和 36.0%±16.1%。较差的预后与“其他中间型”和 11q23 重排 AML 相关(OS 分别为 9.4%±8.7%和 10.7%±9.6%)。复发时间(TTR)小于 1 年的患者 OS 短于 TTR 较长的患者(14.6%±5.4%比 41.1%±11.5%;P=0.006)。在第二次完全缓解(CR2)后进行干细胞移植(SCT)的患者预后优于未行 SCT 的患者(50.9%±11.2%比 7.7%±4.6%;P=0.001)。TTR、风险组、CR2 和 CR2 中的 SCT 是生存的最重要预测因素。

结论

rAML 仍然是一个临床挑战。观察到结局的遗传变异性。大多数 inv(16)患者在复发后得到成功挽救,而 11q23 重排的患者预后较差。仅有三分之一的 t(8;21)rAML 患者存活。HIDC 需要更好地获得 SCT。

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