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微小残留病在不同融合基因背景的急性B淋巴细胞白血病患儿中的临床意义
危彤, 陈晓娟, 张陆阳, 张傲利, 竺晓凡
中国当代儿科杂志 ›› 2020, Vol. 22 ›› Issue (12) : 1279-1285.
PDF(1707 KB)
PDF(1707 KB)
微小残留病在不同融合基因背景的急性B淋巴细胞白血病患儿中的临床意义
Clinical significance of minimal residual disease in B-lineage acute lymphoblastic leukemia pediatric patients with different fusion gene backgrounds
目的 探讨微小残留病（MRD）在不同融合基因背景的急性B淋巴细胞白血病（B-ALL）患儿中的临床意义。方法 回顾性分析2008年1月至2015年4月融合基因阴性（336例）、融合基因ETV6-RUNX1阳性（79例）、融合基因E2A-PBX1阳性（26例）的B-ALL患儿共441例的临床资料。采用流式细胞术检测MRD，分析3组患儿在诱导治疗第15天（TP1）、第33天（TP2）、第12周（TP3）的MRD对预后的影响。结果 在融合基因阴性患儿中，TP1、TP2、TP3时，MRD阳性组总生存（OS）率、无事件生存（EFS）率均显著低于MRD阴性组（P < 0.05），复发率、病死率均显著高于MRD阴性组（P < 0.05）。在ETV6-RUNX1阳性患儿中，仅在TP1时，MRD阳性组OS率、EFS率均显著低于MRD阴性组（P < 0.05），复发率、病死率显著高于MRD阴性组（P < 0.05）。在E2A-PBX1阳性患儿中，TP1、TP2、TP3的MRD阳性组和阴性组的OS率、复发率、病死率差异均无统计学意义（P > 0.05）。结论 在B-ALL患儿中，融合基因阴性患儿的MRD对预后提示意义最为明确，而ETV6-RUNX1阳性患儿和E2A-PBX1阳性患儿的MRD对预后提示意义欠佳。
Objective To study the clinical significance of minimal residual disease (MRD) in B-lineage acute lymphoblastic leukemia (B-ALL) pediatric patients with different fusion gene backgrounds. Methods A retrospective analysis was performed on the medical data of 441 B-ALL children who were treated from January 2008 to April 2015. Among the 441 children, 336 had negative fusion gene, 79 had positive ETV6-RUNX1 fusion gene, and 26 had positive E2A-PBX1 fusion gene. Flow cytometry was used to detect MRD, and the influence of MRD on day 15 (TP1), day 33 (TP2), and week 12 (TP3) of induction therapy on prognosis was analyzed. Results In patients with negative fusion gene, the positive MRD group had significantly lower overall survival (OS) rate and event-free survival (EFS) rate (P < 0.05) and significantly higher recurrence rate and mortality rate at TP1, TP2, and TP3, compared with the negative MRD group (P < 0.05). In patients with positive ETV6-RUNX1, the positive MRD group had significantly lower OS and EFS rates (P < 0.05) and significantly higher recurrence rate and mortality rate (P < 0.05) than the negative MRD group only at TP1. In patients with positive E2A-PBX1, there were no significant differences in the OS rate, recurrence rate, and mortality rate between the positive and negative MRD groups at TP1, TP2, and TP3 (P > 0.05). Conclusions MRD has the most definite prognostic significance in pediatric B-ALL patients with negative fusion gene, while it has unsatisfactory prognostic significance in those with positive ETV6-RUNX1 or positive E2A-PBX1.
急性B淋巴细胞白血病 / 融合基因 / 微小残留病 / 预后 / 儿童
B-lineage acute lymphoblastic leukemia / Fusion gene / Minimal residual disease / Prognosis / Child
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