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.
Eur J Haematol. 2018 Oct;101(4):542-548. doi: 10.1111/ejh.13142. Epub 2018 Aug 31.
The aim of this study was to analyse the clinical characteristics and outcome of children diagnosed with Ph+ ALL.
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).
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.
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.
本研究旨在分析诊断为 Ph+ALL 的儿童的临床特征和结局。
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)。
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。
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。