87例婴儿白血病临床和生物学特征回顾性分析  

Retrospective analysis of clinical and biological characteristics of 87 cases of infantile leukemia

作　　者：缪洁 胡绍燕[1] 闫敬敬 郭爱连 王宇轩 何海龙[1] MIAO Jie;HU Shaoyan;YAN Jingjing;GUO Ailian;WANG Yuxuan;HE Hailong(Department of Hematology and Oncology,Children′s Hospital of Soochow University,Suzhou 215000,China;ZhouKou Central Hospital,Zhoukou 466000,China)

机构地区：[1]苏州大学附属儿童医院血液科,苏州215000 [2]河南省周口市中心医院,周口466000

出　　处：《中国小儿血液与肿瘤杂志》2023年第5期305-311,共7页Journal of China Pediatric Blood and Cancer

摘　　要：目的 通过对婴儿白血病(IL)患儿临床资料、生物学特征、治疗反应及预后情况统计分析,为今后IL的诊治提供参考。方法 回顾分析四个中心87例IL患儿的临床资料,对其性别、年龄、初诊时血常规、生物学特征及疗效预后等探讨分析,采用卡方检验样本率之间的差别,采用Kaplan-Meier曲线分析患儿的生存状况。结果 87例IL患儿中,53例为婴儿急性淋巴细胞白血病(IALL),均为B系,34例为婴儿急性髓细胞性白血病(IAML),亚型以M5及M4较多。诊断时中位年龄为238(172-335)d。初诊时IALL患儿外周血白细胞计数比IAML患儿更高(P=0.013),血红蛋白比IAML患儿更低(P=0.023)。血小板计数比IAML患儿更低(P=0.001)。79例患儿行骨髓染色体检查,IALL和IAML骨髓染色体核型正常与否无差异(P=0.280)。对IALL及IAML患儿MLL伙伴基因对比,发现MLL-AF4在IALL中阳性率较高(P=0.010)。MLL-R基因在IALL中阳性率高于IAML(P=0.012)。MLL-R基因在6-9个月龄患儿中阳性率更高(P=0.025)。MLL-R阳性患儿肝脾肿大多见(P=0.032),外周血白细胞计数更高(P=0.017),骨髓染色体核型多有异常(P=0.004)。87例IL中,33例放弃治疗,54例患儿接受化疗,其中IALL组34例,IAML组20例。两组患儿缓解率、复发率及死亡率比较,发现在IALL组及IAML组中三者差异均无统计学意义。54例接受治疗患儿中16例后期选择造血干细胞移植,38例持续化疗,造血干细胞移植与持续化疗对IL预后无差异(P=1.000)。IL患儿3年EFS为(37.4±9.7)%,3年OS为(53.0±9.3)%。结论 IALL患儿白细胞增高、血小板降低以及血红蛋白降低较IAML表现更明显。MLL-R在IALL中阳性率明显高于IAML,其中MLL-AF4亚型在IALL中最多见。MLL-R阳性多发生于6-9个月龄患儿,与MLL-R阴性相比肝脾肿大多见,外周血白细胞计数较高,骨髓染色体核型多有异常。本研究IL3年EFS及OS均低于国外,提示我们IL治疗水平有待进一步提高。Objective To provide reference for the diagnosis and treatment of infant leukemia(IL)in the future through statistical analysis of clinical data,biological characteristics,treatment response and prognosis of infants leukemia(IL).Methods The clinical data of 87 cases of IL in four centers were retrospectively analyzed,and the gender,age,blood routine test at initial diagnosis,biological characteristics and prognosis of efficacy were analyzed,and the Chi-square test was used to test the difference between sample rates,and the survival status of children was analyzed by Kaplan-Meier curve.Results Among the 87 children with IA,53 had IALL,all of which were B lineage,and 34 had IAML,with more subtypes of M5 and M4.The median age was 238(172,335)days.The peripheral blood white blood cell count of children with IALL was higher than that of children with IAML at the initial diagnosis(P=0.013).The peripheral hemoglobin of children with IALL was lower than that of children with IAML at the initial diagnosis(P=0.023).The platelet count of children with IALL was lower than that of IAML(P=0.001).79 infants had bone marrow chromosome results,here was no difference between the karyotype of IALL and IAML bone marrow(P=0.280).The comparison of MLL partner genes between IALL and IAML showed that MLL-AF4 had a higher positive rate in IALL(P=0.010).The positive rate of MLL-R gene in IALL was higher than that of IAML(P=0.012).The positive rate of MLL-R gene was higher in infants aged 6 to 9 months(P=0.025).Infants with positive MLL-R had hepatosplenomegaly(P=0.032),higher peripheral blood white blood cell count(P=0.017),and bone marrow karyotype abnormalities(P=0.004).Among the 87 cases of IL,33 gave up treatment and 54 children received chemotherapy,including 34 in the IALL group and 20 in the IAML group.Compared with the remission rate,recurrence rate and mortality rate of the two groups,it was found that there was no significant significance in the IALL group and the IAML group.Among 54 infants receiving treatment,16 patients chos

关 键 词：婴儿白血病 MLL基因重排 治疗 预后 

分 类 号：R733.7[医药卫生—肿瘤]