Clinical features and prognosis of childhood B-lineage acute lymphoblastic leukemia expressing the PRAME gene.
10.7499/j.issn.1008-8830.2111108
- Author:
Feng ZHANG
1
;
Ai-Dong LU
1
;
Ying-Xi ZUO
1
;
Ming-Ming DING
1
;
Yue-Ping JIA
1
;
Le-Ping ZHANG
1
Author Information
1. Department of Pediatrics, People's Hospital, Peking University, Beijing 100044, China.
- Publication Type:Journal Article
- Keywords:
Acute lymphoblastic leukemia;
Child;
Minimal residual disease;
Preferentially expressed antigen of melanoma
- MeSH:
Acute Disease;
Antigens, Neoplasm/therapeutic use*;
Child;
Humans;
Neoplasm, Residual/diagnosis*;
Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics*;
Prognosis
- From:
Chinese Journal of Contemporary Pediatrics
2022;24(5):543-549
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVES: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.
