表达<italic>PRAME</italic>基因的儿童急性B淋巴细胞白血病的临床特点及预后分析

doi:10.7499/j.issn.1008-8830.2111108

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Abstract Objective To study the clinical and prognostic significance of the preferentially expressed antigen of melanoma (PRAME) gene in the absence of specific fusion gene expression in children with B-lineage acute lymphoblastic leukemia (B-ALL). Methods A total of 167 children newly diagnosed with B-ALL were enrolled, among whom 70 were positive for the PRAME gene and 97 were negative. None of the children were positive for MLL-r, BCR/ABL, E2A/PBX1, or ETV6/RUNX1. The PRAME positive and negative groups were analyzed in terms of clinical features, prognosis, and related prognostic factors. Results Compared with the PRAME negative group, the PRAME positive group had a significantly higher proportion of children with the liver extending >6 cm below the costal margin (P<0.05). There was a significant reduction in the PRAME copy number after induction chemotherapy (P<0.05). In the minimal residual disease (MRD) positive group after induction chemotherapy, the PRAME copy number was not correlated with the MRD level (P>0.05). In the MRD negative group, there was also no correlation between them (P>0.05). The PRAME positive group had a significantly higher 4-year event-free survival rate than the PRAME negative group (87.5%±4.6% vs 73.5%±4.6%, P<0.05), while there was no significant difference between the two groups in the 4-year overall survival rate (88.0%±4.4% vs 85.3%±3.8%, P>0.05). The Cox proportional-hazards regression model analysis showed that positive PRAME expression was a protective factor for event-free survival rate in children with B-ALL (P<0.05). Conclusions Although the PRAME gene cannot be monitored as MRD, overexpression of PRAME suggests a good prognosis in B-ALL. Citation:Chinese Journal of Contemporary Pediatrics, 2022, 24(5): 543-549

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	ZHANG Feng
	LU Ai-Dong
	ZUO Ying-Xi
	DING Ming-Ming
	JIA Yue-Ping
	ZHANG Le-Ping

Key words： Acute lymphoblastic leukemia Preferentially expressed antigen of melanoma Minimal residual disease Child

Received: 18 November 2021

Cite this article:

ZHANG Feng,LU Ai-Dong,ZUO Ying-Xi et al. Clinical features and prognosis of childhood B-lineage acute lymphoblastic leukemia expressing the PRAME gene[J]. CJCP, 2022, 24(5): 543-549.

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http://www.zgddek.com/EN/10.7499/j.issn.1008-8830.2111108 OR http://www.zgddek.com/EN/Y2022/V24/I5/543

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