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
Khateeb EE, Morgan D. Preferentially expressed antigen of melanoma (PRAME) and Wilms' tumor 1 (WT 1) genes expression in childhood acute lymphoblastic leukemia, prognostic role and correlation with survival[J]. Open Access Maced J Med Sci, 2015, 3(1): 57-62. PMID: 27275197. PMCID: PMC4877789. DOI: 10.3889/oamjms.2015.001.
Paydas S, Tanriverdi K, Yavuz S, et al. PRAME mRNA levels in cases with acute leukemia: clinical importance and future prospects[J]. Am J Hematol, 2005, 79(4): 257-261. PMID: 16044453. DOI: 10.1002/ajh.20425.
Ikeda H, Lethé B, Lehmann F, et al. Characterization of an antigen that is recognized on a melanoma showing partial HLA loss by CTL expressing an NK inhibitory receptor[J]. Immunity, 1997, 6(2): 199-208. PMID: 9047241. DOI: 10.1016/s1074-7613(00)80426-4.