IKZF1基因缺失在急性B淋巴细胞白血病患者中的预后意义

OBJECTIVE: This study aimed to investigate the prognostic significance of IKZF1 gene deletion in patients with acute B lymphoblastic leukemia（B-ALL）. METHODS: The clinical data of 142 patients with B-ALL diagnosed in Nanfang Hospital between March 2016 and September 2019 were analyzed. RESULTS: IKZF1 deletion was found in 36.0％ of the 142 patients with B-ALL, whereas exon 4–7 deletion was found in 44.0％. White blood cell counts were higher in patients with the IKZF1 deletion（52.0％ and 28.3％, P＝0.005）; these patients also experienced worse effects of mid-term induction therapy（40.0％ and 70.7％, P<0.001）and had a higher proportion of Philadelphia chromosome-positive（52.0％ and 21.7％, respectively, P<0.001）. Univariate analysis revealed that the 3-year overall survival rate（OS）and event-free survival rate（EFS）in the IKZF1 deletion group were significantly lower than the IKZF1 wild-type group［（37.1±7.3）％ vs（54.7±5.4）％,（51.8±7.9）％ vs（73.9±4.7）％; P＝0.025, 0.013, respectively］. Multivariable analysis showed that harboring IKZF1 deletion was an adverse factor of EFS and OS（HR＝1.744, 2.036; P＝0.022, 0.020, respectively）. Furthermore, the IKZF1 deletion/chemotherapy group had significantly lower 3-year OS, EFS, and disease-free survival rates than other subgroups. In the IKZF1 deletion cohort, allo-hematopoietic stem cell transplantation（HSCT）significantly improved OS and EFS compared to non-allo-HSCT［（67.9±10.4）％ vs（31.9±11.0）％,（46.6±10.5）％ vs（26.7±9.7）％; P＝0.005, 0.026, respectively］. CONCLUSION: Pediatric-inspired chemotherapy was unable to completely reverse the negative effect of IKZF1 deletion on prognosis. Pediatric-inspired regimen therapy combined with allo-HSCT, in contrast, significantly improved the overall prognosis of IKZF1 deletion B-ALL.