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急性髓系白血病:流行病学特征与生存率分析

Acute Myeloid Leukemia: analysis of epidemiological profile and survival rate.

作者信息

de Lima Mariana Cardoso, da Silva Denise Bousfield, Freund Ana Paula Ferreira, Dacoregio Juliana Shmitz, Costa Tatiana El Jaick Bonifácio, Costa Imaruí, Faraco Daniel, Silva Maurício Laerte

机构信息

Service of Pediatric Oncology, Hospital Infantil Joana de Gusmão, Florianópolis, SC, Brazil.

Service of Pediatric Oncology, Hospital Infantil Joana de Gusmão, Florianópolis, SC, Brazil.

出版信息

J Pediatr (Rio J). 2016 May-Jun;92(3):283-9. doi: 10.1016/j.jped.2015.08.008. Epub 2016 Feb 3.

Abstract

OBJECTIVE

To describe the epidemiological profile and the survival rate of patients with acute myeloid leukemia (AML) in a state reference pediatric hospital.

METHOD

Clinical-epidemiological, observational, retrospective, descriptive study. The study included new cases of patients with AML, diagnosed between 2004 and 2012, younger than 15 years.

RESULTS

Of the 51 patients studied, 84% were white; 45% were females and 55%, males. Regarding age, 8% were younger than 1 year, 47% were aged between 1 and 10 years, and 45% were older than 10 years. The main signs/symptoms were fever (41.1%), asthenia/lack of appetite (35.2%), and hemorrhagic manifestations (27.4%). The most affected extra-medullary site was the central nervous system (14%). In 47% of patients, the white blood cell (WBC) count was below 10,000/mm(3) at diagnosis. The minimal residual disease (MRD) was less than 0.1%, on the 15th day of treatment in 16% of the sample. Medullary relapse occurred in 14% of cases. When comparing the bone marrow MRD with the vital status, it was observed that 71.42% of the patients with type M3 AML were alive, as were 54.05% of those with non-M3 AML. The death rate was 43% and the main proximate cause was septic shock (63.6%).

CONCLUSIONS

In this study, the majority of patients were male, white, and older than 1 year. Most patients with WBC count <10,000/mm(3) at diagnosis lived. Overall survival was higher in patients with MRD <0.1%. The prognosis was better in patients with AML-M3.

摘要

目的

描述一家州立参考儿科医院中急性髓系白血病(AML)患者的流行病学概况和生存率。

方法

临床流行病学、观察性、回顾性、描述性研究。该研究纳入了2004年至2012年间诊断出的15岁以下AML新病例。

结果

在研究的51例患者中,84%为白人;45%为女性,55%为男性。关于年龄,8%小于1岁,47%年龄在1至10岁之间,45%大于10岁。主要体征/症状为发热(41.1%)、乏力/食欲不振(35.2%)和出血表现(27.4%)。最易受影响的髓外部位是中枢神经系统(14%)。47%的患者在诊断时白细胞(WBC)计数低于10,000/mm³。16%的样本在治疗第15天时微小残留病(MRD)小于0.1%。14%的病例发生髓系复发。比较骨髓MRD与生存状态时发现,M3型AML患者中有71.42%存活,非M3型AML患者中有54.05%存活。死亡率为43%,主要直接原因是感染性休克(63.6%)。

结论

在本研究中,大多数患者为男性、白人且大于1岁。大多数诊断时WBC计数<10,000/mm³的患者存活。MRD<0.1%的患者总体生存率更高。AML-M3患者的预后更好。

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