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成人急性早幼粒细胞白血病的疗效:十年经验。

Outcomes in Adult Acute Promyelocytic Leukemia: A Decade Experience.

机构信息

Department of Medical Oncology, Nizam's Institute of Medical Sciences, Hyderabad, India.

Department of Medical Oncology, Nizam's Institute of Medical Sciences, Hyderabad, India.

出版信息

Clin Lymphoma Myeloma Leuk. 2020 Apr;20(4):e158-e164. doi: 10.1016/j.clml.2019.12.011. Epub 2019 Dec 24.

Abstract

BACKGROUND

Acute promyelocytic leukemia (APL), a distinct variant of acute myeloid leukemia (AML), accounts for 10% of AML cases. Over the past decade, APL has emerged from a highly fatal disease to a highly curable one. The published data on outcomes of APL from India are scant. The present study was designed to analyze the clinicopathologic features and outcomes in adult APL.

MATERIALS AND METHODS

The present report is a single institutional, observational, retrospective study, and data of patients diagnosed with APL from 2006 to 2018 were analyzed. All patients with APL, diagnosed by morphology and confirmed by reverse transcriptase polymerase chain reaction (promyelocytic leukemia/retinoic acid receptor alpha) and over 18 years of age, were included in this study. SPSS software v25.1 was used for statistical analysis.

RESULTS

A total of 1396 patients with AML were seen between January 2005 and June 2018, of which 190 (13.6%) had APL. Of these, 111 patients met the inclusion criteria and were analyzed. The median age at presentation was 33 years (range, 19-60 years). The median duration of symptoms before presentation was 15 days (range, 3-180 days). High risk, intermediate risk, and low risk were seen in 43.3%, 41.4%, and 15.3% of patients, respectively. At the end of induction chemotherapy, 88 (79%) patients achieved complete hematologic remission, and 23 (21%) succumbed during induction. The median time to attain complete hematologic remission was 30 days (range, 19-47 days). At a median follow-up of 34 months, the event-free survival and overall survival were 69.3% and 74.7%, respectively. On univariate analysis, bcr3 variant and high-risk category at presentation had significant impact on event-free survival (P = .029 and P = .003, respectively) and overall survival (P ≤ .001 and P = .007, respectively). On multivariate analysis, only high risk at presentation was significant for event-free survival (P = .04) and overall survival (P = .02).

CONCLUSION

APL constituted one-tenth of patients with AML. The majority of patients were high risk at presentation. Sanz high risk category and bcr3 variant at presentation had significant impact on outcomes in APL.

摘要

背景

急性早幼粒细胞白血病(APL)是急性髓系白血病(AML)的一个独特变体,占 AML 病例的 10%。在过去的十年中,APL 已经从一种高度致命的疾病转变为一种高度可治愈的疾病。印度发表的关于 APL 结果的数据很少。本研究旨在分析成人 APL 的临床病理特征和结果。

材料和方法

本报告是一项单机构、观察性、回顾性研究,分析了 2006 年至 2018 年期间诊断为 APL 的患者的数据。所有通过形态学诊断并通过逆转录酶聚合酶链反应(早幼粒细胞白血病/维甲酸受体α)证实且年龄大于 18 岁的 APL 患者均纳入本研究。使用 SPSS 软件 v25.1 进行统计分析。

结果

2005 年 1 月至 2018 年 6 月期间共观察到 1396 例 AML 患者,其中 190 例(13.6%)为 APL。其中,111 例符合纳入标准并进行了分析。发病时的中位年龄为 33 岁(范围,19-60 岁)。发病前症状的中位持续时间为 15 天(范围,3-180 天)。高危、中危和低危患者分别占 43.3%、41.4%和 15.3%。诱导化疗结束时,88 例(79%)患者达到完全血液学缓解,23 例(21%)在诱导期死亡。达到完全血液学缓解的中位时间为 30 天(范围,19-47 天)。中位随访 34 个月时,无事件生存和总生存分别为 69.3%和 74.7%。单因素分析显示,bcr3 变体和发病时的高危类别对无事件生存(P=0.029 和 P=0.003)和总生存(P≤0.001 和 P=0.007)均有显著影响。多因素分析显示,只有发病时的高危因素对无事件生存(P=0.04)和总生存(P=0.02)有显著影响。

结论

APL 占 AML 患者的十分之一。大多数患者在发病时为高危。Sanz 高危类别和发病时的 bcr3 变体对 APL 的结果有显著影响。

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