IKZF1基因突变是成人B系急性淋巴细胞白血病患者预后不良的因素

Ikaros Family Zinc Finger 1 Mutation Is an Adverse Prognostic Factor for Patients with Adult B-Cell Acute Lymphoblastic Leukemia

目的:分析合并IKZF1基因突变的成人B系急性淋巴细胞白血病(B-ALL)患者的预后,探讨异基因造血干细胞移植(allo-HSCT)可否改善患者预后。方法:收集经毛细管电泳法检测骨髓IKZF1基因突变164例成人B-ALL患者的临床资料,分析IKZF1基因突变与成人B-ALL患者的预后关系。结果:164例成人B-ALL患者中IKZF1基因突变阳性80例,阴性84例。80例IKZF1基因突变阳性患者根据诱导化疗缓解后的治疗方式分为移植组(48例)和化疗组(32例)。分析结果显示,移植组中IKZF1突变阳性患者的3年总生存(OS)率、无白血病生存(LFS)率分别为50.3%±8.3%和41.6%±8.5%,化疗组分别为33.7%±12.8%和31.5%±9.5%,均明显低于IKZF1突变阴性患者率(79.5%±7.6%、64.0%±8.4%)和(54.4%±9.9%、40.6%±9.6%)(P<0.05)。IKZF1突变阳性80例患者中移植组3年OS和LFS率(55.3%±7.5%和48.3%±7.6%)明显高于化疗组(32.9%±11.8%和28.4%±10.3%)(P<0.05)。结论:IKZF1基因突变是成人B-ALL预后不良的因素,allo-HSCT可明显提高IKZF1突变阳性成人B-ALL患者的3年OS和LFS率,从而改善其预后。

Objective:To investigate the prognosis of patients with adult B-cell acute lymphoblastic leukemia(B-ALL)accompanied with Ikaros family zinc finger 1(IKZF1)mutation,and to explore the role of allogeneic hematopoietic stem cell transplantation(allo-HSCT)in improving the clinical outcome of patients.Methods:The clinical data of 164 adult B-ALL patients who received IKZF-1 mutation detection by capillary electrophoresis of bone marrow were collected,and the relationship between the IKZF-1 gene mutation and the prognosis of adult B-ALL patients was analyzed.Results:Among 164 adult B-ALL patients,the patients with IKZF-1 mutation(IKZF-1^+)were 80 cases,while the patients without IKZF-1 mutation(IKZF-1^-)were 84 cases.Among 80 IKZF-1 positive patients,according to the treatment method after complete remission these patients were divided into HSCT group(48 cases)and chemotherapy group(32 cases).Analysis showed that the 3-year overall survival(OS)and leukemia-free survival(LFS)rates in the IKZF1^+group were much lower.The OS and LFS rate in transplantation group were 50.3%±8.3%,41.6%±8.5%;the OS and LFS rates in the chemotherapy group were 33.7%±12.8%,31.5%±9.5%,which were lower than those in the IKZF1-group the transplantation group:79.5%±7.6%,64.0%±8.4%;the chemotherapy group:54.4%±9.9%,40.6%±9.6%respectirely(P<0.05).Among 80 B-ALL patients with IKZF-1-mutation,the 3-year OS and LFS rates were significantly higher in the transplantation group(55.3%±7.5%,48.3%±7.6%)than those in the chemotherapy group(32.9%±11.8%,28.4%±10.3%)with IKZF1 mutation(P<0.05).Conclusion:IKZF1 mutation is an adverse prognostic factor for adult B-ALL patients,However,allo-HSCT significantly improves the OS and LFS of patients and also their clinical outcomes.