以面神经麻痹为首发症状的急性白血病患儿的临床分析  

Clinical analysis of acute leukemia in children with facial paralysis as initial symptom

作　　者：胡婉迪 苏淑芳[1] 李白[1] 徐甜甜 马亚南[1] 徐岩[1] 魏林林[1] 刘玉峰[1] Hu Wandi;Su Shufang;Li Bai;Xu Tiantian;Ma Yanan;Xu Yan;Wei Linlin;Liu Yufeng(Department of Hematology and Oncology,Children′s Hospital,First Affiliated Hospital of Zhengzhou University,Children′s Hematology and Oncology Institute of Zhengzhou University,Zhengzhou 450052,Henan Province,China;Department of Pediatrics,Sun Yat-sen Memorial Hospital,Sun Yat-sen University,Guangzhou 510120,Guangdong Province,China)

机构地区：[1]郑州大学第一附属医院儿童医院血液肿瘤科,郑州大学儿童血液肿瘤研究所,郑州450052 [2]中山大学孙逸仙纪念医院儿科,广州510120

出　　处：《国际输血及血液学杂志》2024年第1期46-53,共8页International Journal of Blood Transfusion and Hematology

摘　　要：目的探讨以面神经麻痹为首发症状的急性白血病(AL)患儿的临床特征、治疗及预后。方法选择2016年8月至2021年8月郑州大学第一附属医院儿童血液肿瘤科收治的以面神经麻痹为首发症状的11例AL患儿为研究对象,其中男性患儿为8例,女性为3例。采用回顾性研究方法,分析本组患儿的临床特征,包括一般临床资料、主要检查结果、治疗及预后情况等。采用Kaplan-Meier法绘制AL患儿的生存曲线。采用log-rank检验比较不同临床特征AL患儿的生存率。采用Cox比例风险回归模型,对可能影响AL患儿的预后因素进行多因素分析。本研究通过郑州大学第一附属医院医学伦理委员会审查批准(批准文号:2021-KY-0594)。结果①本组11例AL患儿中,急性淋巴细胞白血病(ALL)患儿为3例,急性髓细胞白血病(AML)为8例;首发症状均为面神经麻痹;发病至确诊中位时间为11.0 d(8.0,24.0 d);出现伴随症状者为10例,首诊科室系非血液科者为6例。②本组11例AL患儿的血常规和骨髓细胞形态学结果均显示异常,脑脊液细胞学检查、头颅磁共振成像(MRI)结果异常者分别为3、5例。急性T淋巴细胞白血病(T-ALL)、AML-M2、AML-M5患儿分别为3、7和1例。融合基因、基因突变、染色体核型检测结果阳性者分别为7、8和8例。③本组11例AL患儿中,既往应用糖皮质激素者为5例,化疗后达完全缓解(CR)者为10例,其中复发者为7例。④中位随访23.3个月(8.3,31.9个月)时,死亡患儿为8例。本组11例患儿的3年总体生存(OS)率为27.3%。多因素Cox比例风险回归模型分析结果显示,患儿既往应用糖皮质激素为以面神经麻痹为首发症状的AL患儿OS率的独立危险因素(HR=12.170,95%CI:1.373~107.847,P=0.025)。结论以面神经麻痹为首发症状的AL患儿早期临床症状不典型,发病至确诊间隔时间长,预后差。对于病因不明或治疗效果差的面神经麻痹患儿,需首先排除AL可能,并且Objective To investigate clinical features,treatment and prognosis of acute leukemia(AL)in children with facial paralysis as the initial symptom.Methods From August 2016 to August 2021,a total of 11 AL children with facial paralysis as the initial symptom in the Department of Pediatric Hematology and Oncology,the First Affiliated Hospital of Zhengzhou University were selected as research subjects.Among them,8 cases were male and 3 cases were female.A retrospective study was conducted to collect clinical data of these children,including general clinical data,main examination results,treatment and prognosis.Kaplan-Meier method was used to draw survival curves for AL children.The log-rank test was used to compare survival rates of AL children with different clinical characteristics.Use the Cox proportional hazard regression model for multivariate analysis of prognostic factors in those AL children.This study was reviewed and approved by the Ethics Committee of First Affiliated Hospital of Zhengzhou University(Approval No.2021-KY-0594).Results①Of the 11 AL children,3 cases were acute lymphoblastic leukemia(ALL)and 8 cases were acute myeloid leukemia(AML),all of them had facial paralysis as the initial symptom.The median duration from onset to diagnosis was 11.0 d(8.0,24.0 d),and accompanying signs and symptoms were present in 10 cases.The first department of diagnosis was non-hematology in 6 cases.②All 11 AL children had abnormal results of peripheral blood routine examination and bone marrow cell morphology examination.The numbers of AL children who had abnormal results of cerebrospinal fluid cytology and cranial magnetic resonance imaging(MRI)were 3 cases and 5 cases,respectively.T-cell acute lymphoblastic leukemia(T-ALL),AML-M2 and AML-M5 children were 3 cases,7 cases,and 1 case,respectively.There were 7 cases,8 cases and 8 cases with positive fusion gene,gene mutation and chromosomal karyotype test,respectively.③Of the 11 AL children,there were 5 cases who had used glucocorticoids before.After chemotherapy

关 键 词：面神经麻痹 面瘫 白血病浸润 白血病 髓样 急性 中枢神经系统肿瘤 预后 儿童 

分 类 号：R733.71[医药卫生—肿瘤]