Clinical features and prognosis of high hyperdiploid childhood acute lymphoblastic leukemia: a multicenter retrospective analysis in Fujian Province, China / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features and prognosis of high hyperdiploid (HHD) childhood acute lymphoblastic leukemia (ALL).@*METHODS@#A retrospective analysis was performed on the medical data of 1 414 children who were newly diagnosed with ALL and were admitted to five hospitals in Fujian Province of China from April 2011 to December 2020. According to karyotype, they were divided into two groups: HHD (n=172) and non-HHD (n=1 242). The clinical features and treatment outcome were compared between the two groups, and the factors influencing the prognosis were further explored.@*RESULTS@#Among the 1 414 children with ALL, 172 (12.16%) had HHD. Compared with the non-HHD group, the HHD group had significantly lower proportions of children with risk factors for poor prognosis at diagnosis (age of onset ≥10 years or <1 year, white blood cell count ≥50×109/L, and T-cell phenotype) or positive fusion genes (TEL-AML1, BCR-ABL1, E2A-PBX1, and MLL gene rearrangement) (P<0.05). The HHD group had a significantly higher proportion of children with minimal residual disease (MRD) <0.01% at the end of induction chemotherapy (P<0.05). The 10-year event-free survival (EFS) rate and overall survival (OS) rate in the HHD group were significantly higher than those in the non-HHD group (P<0.05). The univariate analysis showed that the number of chromosomes of 58-66, trisomy of chromosome 10, trisomy of chromosome 17, bone marrow MRD <1% on day 15 or 19 of induction chemotherapy, and bone marrow MRD <0.01% on day 33 or 46 of induction chemotherapy were associated with a higher EFS rate (P<0.05), and trisomy of chromosome 10 was associated with a higher OS rate (P<0.05). The multivariate Cox analysis showed that trisomy of chromosome 17 was closely associated with a high EFS rate (P<0.05).@*CONCLUSIONS@#The ALL children with HHD have few risk factors for poor prognosis at diagnosis and often have good prognosis. The number of chromosomes and trisomy of specific chromosomes are associated with prognosis in these children.

Prognostic significance of high hyperdiploid and triploid/tetraploid acute myeloid leukemia / 临床检验杂志

Objective@#To investigate the biological characteristics of high hyperdiploid and triploid/tetraploid acute myeloid leukemia (HH/TT-AML) and its relationship with prognosis. @*Methods@#The clinical data of 28 patients with newly diagnosed HH/TT-AML during March 2006 and June 2017 were retrospectively analyzed, and the factors influencing prognosis were analyzed by the Kaplan-Meier method. @*Results@#The karyotypes of HH/TT-AML patients were mainly 49 chromosomes, accounting for 39.3% (11/28), followed by 50-55 chromosomes, accounting for 32.1% (9/28). The karyotypes of high hyperdiploid acute myeloid leukemia (HH-AML) patients were more likely to be +8 (77.3%, 17/22) or +21 (54.5%, 12/22). The survival analysis showed that the overall survival rate of HH/TT-AML patients with -5/5q-, -7/7q-, -17/der(17p) or der(3q) was significantly lower than that without these abnormalities (4.1 months vs 10.1 months,P＜0.05). There was no significant difference in the overall survival rate between triploid/tetraploid acute myeloid leukemia (TT-AML) patients and HH-AML patients (8.4 months vs 7.2 months,P＞0.05). The overall survival rate of the patients with allogeneic hematopoietic stem cell transplantation was significantly longer than that with chemotherapy alone (25.4 months vs 4.1 months,P＜0.01). @*Conclusion@#HH/TT AML patients are highly heterogeneous. The identification of poor prognosis-related chromosome abnormalities is helpful for the stratification of prognosis. The overall survival time of these patients is short. Early allogeneic hematopoietic stem cell transplantation after remission by combination chemotherapy can significantly improve the prognosis of these patients.