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新诊断的急性髓系白血病合并白细胞增多症患儿的临床和生物学特征及治疗结果

Clinical and biologic features and treatment outcome of children with newly diagnosed acute myeloid leukemia and hyperleukocytosis.

作者信息

Inaba Hiroto, Fan Ying, Pounds Stanley, Geiger Terrence L, Rubnitz Jeffrey E, Ribeiro Raul C, Pui Ching-Hon, Razzouk Bassem I

机构信息

Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.

出版信息

Cancer. 2008 Aug 1;113(3):522-9. doi: 10.1002/cncr.23581.

Abstract

BACKGROUND

Acute myeloid leukemia (AML) with hyperleukocytosis often is associated with early complications. To the authors' knowledge, no recently published study has evaluated the management and clinical course in this regard, especially in relation to pediatric patients.

METHODS

The authors reviewed 579 patients with newly diagnosed pediatric AML who were treated at St. Jude Children's Research Hospital from 1968 to 2002 and carefully examined 106 patients with initial leukocyte counts > or = 100 x 10(9)/L and French-American-British (FAB) AML subtypes other than M3. These patients with hyperleukocytosis were divided into 2 groups-'before' (early period; 70 patients) and 'after' (late period; 36 patients) the initiation of the AML-83 protocol-to address potential differences in supportive measures (including leukoreduction).

RESULTS

Forty-five patients (42.5%) had early complications that were associated strongly with M4 and M5 FAB subtypes and had higher initial leukocyte counts than the patients without complications. Early deaths were less common in the late period (2.8%) than in the early period (22.9%; P = .01), although the incidence of early complications was similar. The late period was associated with a shorter time for referral (P = .0018), a longer time from admission to chemotherapy initiation (P < .0001), and lower white blood cell counts at chemotherapy initiation (P < .0001). In the late period, patients with or without hyperleukocytosis had similar complete remission rates. However, those with hyperleukocytosis had a lower postremission 10-year event-free survival rate (21.2% vs 41.7%; P = .0228).

CONCLUSIONS

With improved management, including supportive care, early mortality in patients with AML and hyperleukocytosis decreased remarkably in the more recent period. However, better postremission treatment is required to improve long-term survival.

摘要

背景

伴有白细胞增多症的急性髓系白血病(AML)常伴有早期并发症。据作者所知,最近没有发表的研究评估过这方面的治疗及临床病程,尤其是针对儿科患者。

方法

作者回顾了1968年至2002年在圣裘德儿童研究医院接受治疗的579例新诊断的儿科AML患者,并仔细研究了106例初始白细胞计数≥100×10⁹/L且法美英(FAB)AML亚型非M3的患者。这些白细胞增多症患者被分为两组——AML-83方案启动“之前”(早期;70例患者)和“之后”(晚期;36例患者)——以探讨支持性措施(包括白细胞去除术)方面的潜在差异。

结果

45例患者(42.5%)出现早期并发症,这些并发症与FAB M4和M5亚型密切相关,且初始白细胞计数高于无并发症的患者。尽管早期并发症的发生率相似,但晚期的早期死亡率(2.8%)低于早期(22.9%;P = 0.01)。晚期的转诊时间较短(P = 0.0018),从入院到开始化疗的时间较长(P < 0.0001),化疗开始时的白细胞计数较低(P < 0.0001)。在晚期,有或无白细胞增多症的患者完全缓解率相似。然而,白细胞增多症患者缓解后的10年无事件生存率较低(21.2%对41.7%;P = 0.0228)。

结论

随着包括支持性护理在内的管理改善,AML伴白细胞增多症患者的早期死亡率在最近时期显著下降。然而,需要更好的缓解后治疗来提高长期生存率。

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