Clinical characterization and prognosis of T cell acute lymphoblastic leukemia with high CRLF2 gene expression in children
Clinical characterization and prognosis of T cell acute lymphoblastic leukemia with high CRLF2 gene expression in children
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CRLF2基因高表达儿童T细胞急性淋巴细胞白血病的临床特征及预后
DOI：
10.1371/journal.pone.0224652
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发表时间：
2019-12-12
期刊：
影响因子：
3.7
通讯作者：
Xiao, Jianwen
中科院分区：
文献类型：
--
作者：
Wang, Mingmin;Wen, Jinquan;Xiao, Jianwen
据报道，CRLF2基因过度表达与儿童B细胞急性淋巴细胞白血病（B-ALL）不良结局相关，但CRLF2基因过度表达在儿童T细胞ALL（T-ALL）中的发病率、临床特征和结局尚未系统分析。本研究检测了2015年2月至2018年6月在重庆医科大学儿童医院和咸阳市儿童医院就诊的63例儿童T-ALL患者的CRLF2 mRNA表达水平以及临床和实验室参数。患者按照改良的圣裘德TXV ALL方案进行治疗，并评估入组患者的早期治疗反应（骨髓涂片和MRD水平）和预后。在 21/63 (33.33%) 的患者中检测到 CRLF2 过度表达。 CRLF2高表达患者与CRLF2低表达患者的临床或实验室参数（包括性别、年龄、初始WBC计数、纵隔受累发生率、核型异常及融合基因等）差异无统计学意义（P>0.05）。一名患者死于肿瘤溶解综合征和肾功能衰竭，并在 62 名患者缓解的第 19 天（TP1）监测治疗反应。一名患者因家庭决定退出治疗，61 名患者在缓解第 46 天（TP2）接受治疗反应评估。 CRLF2高表达患者与CRLF2低表达患者TP1、TP2治疗反应差异无统计学意义（P>0.05）。 2018年10月后，61名可评估患者中有12名患者复发（复发率：19.67%），3名患者死于化疗，治疗相关死亡率（TRM）为4.92%。 1例患者发生继发肿瘤。 61 名可评估患者和 58 名未接受 TRM 的患者的 3 年预期 EFS 率为 54.1 +/- 11.2% 和 77.7 +/- 6.6%。 CRLF2 低表达患者的 EFS 持续时间比 CRLF2 高表达患者更长（61 名可评估患者：35.91 +/- 2.38 个月 vs 23.43 +/- 2.57 个月；58 名无 TRM 患者：37.86 +/- 2.08 个月 vs 24.55 +/- 2.43 个月，P
It has been reported that overexpression of the CRLF2 gene is associated with poor outcomes in pediatric B cell acute lymphoblastic leukemia (B-ALL), but the incidence rates, clinical characteristics and outcomes of CRLF2 gene overexpression in pediatric T cell ALL (T-ALL) have not been systematically analyzed. In this study, CRLF2 mRNA expression levels and clinical and laboratory parameters in 63 pediatric T-ALL patients were detected at the Children's Hospital of Chongqing Medical University and Children's Hospital of Xianyang between February 2015 and June 2018. The patients were treated according to the modified St. Jude TXV ALL protocol, and early treatment responses (bone marrow smear and MRD level) and prognoses in the enrolled patients were assessed. CRLF2 overexpression was detected in 21/63 (33.33%) patients. Statistical differences were not found for clinical or laboratory parameters (including sex, age, initial WBC count, incidence mediastinal involvement, abnormal karyotype and fusion genes) between patients with high CRLF2 expression and patients with low expression of CRLF2 (P>0.05). One patient died of tumor lysis syndrome and renal failure, and the treatment response was monitored on day 19 (TP1) of remission in 62 patients. One patient quit treatment because of family decisions, and 61 patients underwent treatment response evaluation on day 46 (TP2) of remission. Significant differences were not found between patients with high CRLF2 expression and patients with low CRLF2 expression in terms of the treatment responses at TP1 or TP2 (P>0.05). Following October 2018, 12 patients among the 61 evaluable patients relapsed (relapse rate: 19.67%), 3 patients died from chemotherapy, and the treatment-related mortality (TRM) rate was 4.92%. Secondary tumors occurred in 1 patient. The 3-year prospective EFS rate was 54.1 +/- 11.2% and 77.7 +/- 6.6% for the 61 evaluable patients and 58 patients without TRM. Patients with low CRLF2 expression had longer EFS durations than patients with high CRLF2 expression (61 evaluable patients: 35.91 +/- 2.38 months vs 23.43 +/- 2.57 months; 58 patients without TRM: 37.86 +/- 2.08 months vs 24.55 +/- 2.43 months, P