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  

Clinical Features and Prognosiss of Pediatric Acute Lymphoblastic Leukemia with Hyperleukocytosis

作　　者：李海金 李慧园 刘新妙 田玥 李娜 段正铖 田新[1] LI Haijin;LI Huiyuan;LIU Xinmiao;TIAN Yue;LI Na;DUAN Zhengcheng;TIAN Xin(Dept.of Hematology and Oncology,Kunming Children’s Hospital/Children’s Hospital Affiliated to Kunming Medical University,Kunming Yunnan 650228;Dept.of Pediatric,Kunming Maternal and Child Health Hospital,Kunming Yunnan 650031,China)

机构地区：[1]昆明市儿童医院/昆明医科大学附属儿童医院血液肿瘤科,云南昆明650228 [2]昆明市妇幼保健院儿科,云南昆明650031

出　　处：《昆明医科大学学报》2024年第7期105-112,共8页Journal of Kunming Medical University

基　　金：云南省竺晓凡专家工作站资助项目(202105AF150070)。

摘　　要：目的探讨儿童高白细胞性急性淋巴细胞白血病(HL-ALL)的临床特征及预后影响因素。方法回顾性分析昆明市儿童医院血液肿瘤科2019年1月至2022年7月收治的427例初诊ALL患儿临床资料,以初诊白细胞计数(WBC)100×10^(9)/L为界限,分为高白组92例与非高白组335例,比较2组间的临床特征、主要实验室检查指标、总生存时间(OS)、无事件生存时间(EFS)等。结果427例ALL患儿中,男性250例,女性177例,男女比例1.41∶1,高白组92例(21.5%),非高白组335例(78.5%)。与非高白组相比,高白组患儿年龄<1岁和>10岁、T淋巴细胞白血病(T-ALL)、中重度肝脾肿大、危险度为高危的比例更高(P<0.05),初诊幼稚细胞百分比、血尿酸(UA)水平、血乳酸脱氢酶(LDH)水平更高(P<0.05),初诊血小板计数(PLT)水平以及合并超二倍体的比例低(P<0.05)。高白组的3 a EFS及OS均低于非高白组,多因素回归分析显示,初诊白细胞计数≥100×10^(9)/L、年龄>10岁、年龄<1岁、T-ALL、高危、D15 MRD阳性、D33 MRD阳性、CNSL、MLL重排阳性、MEF2D重排阳性是影响ALL患儿EFS率及OS率的独立危险因素(P<0.05)。结论HL-ALL患儿初诊时普遍存在显著外周血幼稚百分比更高,血UA及血LDH升高,并且常伴有发病年龄<1岁或>10岁、T-ALL、肝脾肿大、高危,高白组患儿早期治疗反应欠佳,预后不良。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 100×10^(9)/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≥100×10^(9)/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,TALL,hepatosplenomegaly,and high risk.The early treatment response and prognosis of the children with HL-ALL are poor.

关 键 词：急性淋巴细胞白血病 高白细胞性 儿童 

分 类 号：R733[医药卫生—肿瘤]