儿童高白细胞性急性淋巴细胞白血病的临床特征及预后分析OACSTPCD

Objective To investigate the clinical features and prognostic factors of pediatric acute lymphoblastic leukemia with hyperleukocytosis(HL-ALL).Methods The clinical data of 427 newly diagnosed ALL children admitted to the Department of Hematology and Oncology of Kunming Children's Hospital from January 2019 to July 2022 were retrospectively analyzed.According to the initial white blood cell count(WBC)of 100×109/L,the children were divided into the hyperleukocytosis group and the non-hyperleukocytosis group.The clinical characteristics,major laboratory tests,overall survival(OS)and event-free survival(EFS)were compared between the two groups.Results There were 250 males and 177 females,with a male-to-female ratio of 1.41∶1.There were 92 cases(21.5%)in the hyperleukocytosis group and 335 cases(78.5%)in the non-hyperleukocytosis group.Compared with the non-hyperleukocytosis group,the hyperleukocytosis group had significantly higher proportions of children aged<1 year and>10 years,T lymphoblastic leukemia(T-ALL),moderate to severe hepatosplenomegaly,and high-risk risk(P<0.05),as well as significantly higher percentage of immature cells at initial diagnosis,serum uric acid(UA)level,and lactate dehydrogenase(LDH)level(P<0.05).The platelet count(PLT)and the proportion of hyperdiploidy at diagnosis were lower(P<0.05).The 3-year EFS and OS of the hyperleukocytosis group were lower than those of the non-hyperleukocytosis group.WBC≥100×109/L at diagnosis,age>10 years,age<1 year,T-ALL,high-risk,D15 MRD positive,D33 MRD positive,CNSL,MLL rearrangement,and MEF2D rearrangement were independent risk factors for EFS and OS rates in children with ALL(P<0.05).Conclusion The children with HL-ALL usually have a higher percentage of peripheral blood immature cells,elevated serum UA and LDH,and are often accompanied by age of onset<1 or>10 years old,T-ALL,hepatosplenomegaly,and high risk.The early treatment response and prognosis of the children with HL-ALL are poor.