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2005 年至 2017 年波兰 Ph 阳性急性淋巴细胞白血病患儿的临床特征及治疗效果分析。

Clinical characteristics and analysis of treatment result in children with Ph-positive acute lymphoblastic leukaemia in Poland between 2005 and 2017.

机构信息

Department of Paediatric Haematology, Oncology and Transplantology, Medical University of Lublin, Lublin, Poland.

Department of Paediatric Haematology, Oncology and Transplantology, Genetic Diagnostic Laboratory, University Children's Hospital, Lublin, Poland.

出版信息

Eur J Haematol. 2018 Oct;101(4):542-548. doi: 10.1111/ejh.13142. Epub 2018 Aug 31.

Abstract

OBJECTIVE

The aim of this study was to analyse the clinical characteristics and outcome of children diagnosed with Ph+ ALL.

MATERIAL AND METHODS

A total of 2591 newly diagnosed ALL children were treated in Poland between the years 2005 and 2017. Of those, 44 were diagnosed with Ph(+) ALL. The patients were treated according to protocols: ALL IC-BFM 2002 and 2009 (26 patients), EsPhALL (12 patients), initially ALL IC-BFM and then EsPhALL (6 patients).

RESULTS

The median of follow-up in the observed group was 3 years. Overall survival (OS) and event-free survival (EFS) of Ph+ ALL group were 0.73 and 0.64. OS and EFS of patients after HSCT were 0.78 and 0.66, while without HSCT were 0.6 and 0.6, P = 0.27 and 0.63. OS was 0.8 for patients treated with chemotherapy plus imatinib and 0.61 for chemotherapy alone, P = 0.22, while EFS was 0.66 (imatinib therapy) and 0. 61 (without imatinib), P = 0.41.

CONCLUSION

Our study suggests that adding imatinib to intensive chemotherapy seems to improve outcome. However, this study was limited by a small number of patients and a variety of chemotherapy regimens with or without imatinib.

摘要

目的

本研究旨在分析诊断为 Ph+ALL 的儿童的临床特征和结局。

材料与方法

2005 年至 2017 年期间,波兰共收治了 2591 例新诊断的 ALL 儿童,其中 44 例被诊断为 Ph(+)ALL。患者接受了以下方案治疗:ALL IC-BFM 2002 方案和 2009 方案(26 例)、EsPhALL 方案(12 例)、初始 ALL IC-BFM 方案随后改为 EsPhALL 方案(6 例)。

结果

观察组的中位随访时间为 3 年。Ph+ALL 组的总生存率(OS)和无事件生存率(EFS)分别为 0.73 和 0.64。HSCT 后患者的 OS 和 EFS 分别为 0.78 和 0.66,而未行 HSCT 患者的 OS 和 EFS 分别为 0.6 和 0.6,P 值分别为 0.27 和 0.63。接受化疗联合伊马替尼治疗的患者 OS 为 0.8,单独接受化疗的患者 OS 为 0.61,P 值为 0.22,而 EFS 分别为 0.66(伊马替尼治疗)和 0.61(无伊马替尼),P 值为 0.41。

结论

我们的研究表明,在强化化疗中加入伊马替尼似乎可以改善预后。然而,本研究受到患者数量少和有无伊马替尼的多种化疗方案的限制。

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