Prognostic analysis of children with Philadelphia chromosome-like acute lymphoblastic leukemia common genes.
10.3760/cma.j.cn112140-20221005-00853
- Author:
Wan Di HU
1
;
Bai LI
1
;
Shu Fang SU
1
;
Yu Feng LIU
1
;
Wei LIU
2
;
Wen Lin ZHANG
3
;
Wen Li ZUO
3
;
Run Hong YU
4
Author Information
1. Department of Hematology and Oncology, Children's Hospital, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
2. Department of Hematology and Oncology, Henan Children's Hospital, Zhengzhou 450018, China.
3. Department of Pediatric Hematology and Oncology, Henan Cancer's Hospital, Zhengzhou 450008, China.
4. Department of Hematology, Henan Provincial People's Hospital, Zhengzhou 450003, China.
- Publication Type:Journal Article
- MeSH:
Male;
Female;
Humans;
Child;
Prognosis;
Philadelphia Chromosome;
Retrospective Studies;
Receptor, Platelet-Derived Growth Factor beta/genetics*;
Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy*;
Neoplasm, Residual
- From:
Chinese Journal of Pediatrics
2023;61(5):446-452
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To summarize the clinical data and prognosis of children with Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) common genes. Methods: This was a retrospective cohort study.Clinical data of 56 children with Ph-like ALL common gene cases (Ph-like ALL positive group) treated from January 2017 to January 2022 in the First Affiliated Hospital of Zhengzhou University, Henan Children's Hospital, Henan Cancer's Hospital and Henan Provincial People's Hospital were collected, 69 children with other high-risk B cell acute lymphoblastic leukemia (B-ALL) at the same time and the same age were selected as the negative group. The clinical characteristics and prognosis of two groups were analyzed retrospectively. Comparisons between groups were performed using Mann-Whitney U test and χ2 test. Kaplan-Meier method was used for survival curve, Log-Rank test was used for univariate analysis, and the Cox regression model was used for multivariate prognosis analysis. Results: Among 56 Ph-like ALL positive patients, there were 30 males and 26 females, and 15 cases were over 10 years old. There were 69 patients in Ph-like ALL negative group. Compared with the negative group, the children in positive group were older (6.4 (4.2, 11.2) vs. 4.7 (2.8, 8.4) years), and hyperleukocytosis (≥50×109/L) was more common (25% (14/56) vs. 9% (6/69)), the differences were statistically significant (both P<0.05). In the Ph-like ALL positive group, 32 cases were positive for IK6 (1 case was co-expressed with IK6 and EBF1-PDGFRB), 24 cases were IK6-negative, of which 9 cases were CRLF2 positive (including 2 cases with P2RY8-CRLF2, 7 cases with CRLF2 high expression), 5 cases were PDGFRB rearrangement, 4 cases were ABL1 rearrangement, 4 cases were JAK2 rearrangement, 1 case was ABL2 rearrangement and 1 case was EPOR rearrangement. The follow-up time of Ph-like ALL positive group was 22 (12, 40) months, and 32 (20, 45) months for negative group. The 3-year overall survival (OS) rate of positive group was significantly lower than the negative group ((72±7) % vs. (86±5) %, χ2=4.59, P<0.05). Compared with the 24 IK6-negative patients, the 3-year event free survival (EFS) rate of 32 IK6 positive patients was higher, the difference was statistically significant ((88±9) % vs. (65±14) %, χ2=5.37, P<0.05). Multivariate Cox regression analysis showed that the bone marrow minimal residual disease (MRD) not turning negative at the end of first induction (HR=4.12, 95%CI 1.13-15.03) independent prognostic risk factor for patient with Ph-like ALL common genes. Conclusions: Children with Ph-like ALL common genes were older than other high-risk B-ALL patients at diagnosis, with high white blood cells and lower survival rate. The bone marrow MRD not turning negative at the end of first induction were independent prognostic risk factor for children with Ph-like ALL common gene.
