doi:10.3969/j.issn.1000-3606.2018.01.011通信作者：张乐萍 电子信箱：zhangleping@伴TCF3-PBX1阳性的儿童急性淋巴细胞白血病的临床特点及预后王 毓 张乐萍 陆爱东 左英熹 吴 珺北京大学人民医院儿科（北京 100044）摘要： 目的 探讨TCF3-PBX1阳性急性淋巴细胞白血病（ALL ）的临床特征、疗效及预后影响因素。
方法 回顾性分析2006年8月至2015年8月收治的29例初诊为TCF3-PBX1阳性ALL 患儿的临床资料，应用实时定量反转录聚合酶链式反应定期监测TCF3-PXB1融合基因表达水平，分析影响TCF3-PBX1阳性ALL 患儿预后的影响因素。
结果 29例患儿，男16例、女13例，中位年龄8岁（9个月~16岁）。
免疫表型以前B 细胞型最多见（58.6%）；染色体核型分析显示以不平衡易位多见（41.4%）。
29例患儿治疗第33天完全缓解率达100%，但微小残留病灶未完全转阴，3例患儿复发均为微小残留病（MRD ）未转阴者。
Cox 多因素回归分析显示，年龄是影响5年总生存率（OS ）的独立危险因素（P <0.05）。
5年OS 及无病生存率（DFS ）分别为（82±8）%、（81±7）%。
结论 伴TCF3-PBX1阳性的儿童ALL 是一类具有高度异质性疾病，治疗完全缓解率高，远期疗效好，对患儿进行危险度分层同时行个体化治疗是提高治愈率的关键。
关键词： 急性淋巴细胞白血病； 微小残留病； 预后； 儿童Clinical characteristics and prognosis of childhood TCF3-PBX1 positive acute lymphoblastic leukemia WANG Yu ，ZHANG Leping ，LU Aidong ，ZUO Yingxi ，WU Jun (Department of Pediatrics, Peking University People’s Hospital, Beijing 100044, China)Abstract: Objective To explore the clinical characteristics and relevant factors affecting treatment and prognosis of TCF3-PBX1 positive acute lymphoblastic leukemia (ALL). Methods The clinical data of 29 children with newly diagnosed TCF3-PBX1 positive ALL from August 2006 to August 2015 were analyzed retrospectively. The expression level of TCF3-PXB1 fusion gene was monitored by regular quantitative reverse transcription polymerase chain reaction. The factors influencing prognosis in children with TCF3-PBX1 positive ALL were analyzed. Results There were 29 children (16 males and 13 females) with a median age of 8 years (9 months to 16 years). The most common immunophenotype was pre-B cell type (pre-B) (58.6%). The karyotype analysis showed that unbalanced translocation was more common (41.4%). The complete remission rate was 100% on thirty-third day in 29 children, but the minimal residual disease (MRD) was not completely negative. Three cases were relapsed, all of whom were MRD positive. Cox multivariate regression analysis showed that age was an independent risk factor for 5 year overall survival (P <0.05). The 5 year overall survival rate and disease-free survival rate were (82±8)% and (81±7)% respectively. Conclusions Childhood TCF3-PBX1 positive ALL is a highly heterogeneous disease with high rate of complete remission and good long-term efficacy. The risk stratification and individualized treatment is the key to improve the cure rate.Key words: acute lymphoblastic leukemia; minimal residual disease; prognosis; child急性淋巴细胞白血病（acute lymphoblastic leukemia ，ALL ）约占儿童急性白血病的75%~80%，是儿童恶性肿瘤中最常见的类型[1]。
白血病•淋巴瘤2020年12月第29卷第12期Journal cf Leukemia&Lymphoma,December2020,Vol.29.No.12・727・•论著•急性T淋巴细胞白血病23例临床分析王莹陈饪张苏江上海交通大学医学院附属瑞金医院北部院区血液科201801通信作者：张苏江，Email:zbruce.*****************【摘要】目的探讨急性T淋巴细胞白血病患者的近期疗效、预后及预后相关因素。
方法回顾性分析2013年1月至2018年12月上海交通大学医学院附属瑞金医院北部院区收治的23例初发急性T淋巴细胞白血病患者临床资料，并结合文献对其临床特征、治疗及转归进行总结。
结果23例患者中，男性14例，女性9例，中位年龄32岁(14~58岁)。
诱导治疗采用标准VDPCP方案，总有效率为60.9%(14/23),完全缓解率为47.8%(11/23),部分缓解率为13.0%(3/23)；中位无进展生存时间为11.5个月(0〜66.0个月)冲位总生存时间为15.4个月(2.0〜66.0个月)。
与诱导缓解相关的可评价预后因素包括年龄、初发时白细胞计数、NOTCH1基因突变、染色体核型及疾病特殊类型，但差异均无统计学意义(均P>0.05)o结论急性T淋巴细胞白血病患者预后较差，初次诱导缓解率低，无进展生存时间和总生存时间短。
【关键词】白血病,T细胞；治疗结果；预后DOI：10.3760/115356-20200531-00147T-cell acute lymphocytic leukemia:clinical analyses of23casesWang Ying,Chen Yu,Zhang SujiangDepartment of Hematology,North District of Ruijin Hospital Affiliated to Medical School of Shanghai JiaotongUniversity,Shanghai201801,ChinaCorresponding author:Zhang Sujiang,Email:zbruce******************[Abstract]Objective To investigate the effectiveness,prognosis and prognostic factors of patientswith T-cell acute lymphocytic leukemia.Methods The clinical data of23patients with newly diagnosedT-cell acute lymphocytic leukemia from January2013to December2018in North District of Ruijin HospitalAffiliated to Medical School of Shanghai Jiaotong University were retrospectively analyzed,and their clinicalcharacteristics,treatment and prognosis were summarized combined with literature.Results Among23patients,14were males and9were females,with a median age of32years old(14-58years old).Theinduction therapy adopted standard VDPCP regimen.The overall response rate was60.9%(14/23),thecomplete remission rate was47.8%(11/23),the partial remission rate was13.0%(3/23),the medianprogression-free survival time was11.5months(0-66.0months),and the median overall survival time was15.4months(2.0-66.0months).The evaluable prognostic factors associated with induction remission includedage,white blood cell count at the time of onset,NOTCH1gene mutation,chromosome karyotype,and specifictypes of disease,but the differences were not statistically significant(all P>0.05).Conclusion Theprognosis of patients with T-cell acute lymphocytic leukemia is poor,the remission rate of initial in d uction islow,the progression-free survival time is short,and the overall survival time is short as well.[Key words]Leukemia,T-cell;Treatment outcome;PrognosisDOI:10.3760/115356-20200531-00147扫码阅读电子版急性T淋巴细胞白血病（T-ALL）约占急性淋巴细胞白血病的25%,总体发病率不高。
·89JOURNAL OF RARE AND UNCOMMON DISEASES, MAY. 2023,Vol.30, No.5, Total No.166【第一作者】尚 楠，男，主管检验技师，主要研究方向：临床检验方面的。
E-mail：*****************【通讯作者】尚 楠·论著·血清白细胞介素-6和降钙素原水平与急性淋巴细胞白血病患儿预后的关系尚 楠* 铁 峰 刘鹏飞开封市儿童医院检验科 (河南 开封 475000)【摘要】目的 探讨血清白细胞介素-6(IL-6)和降钙素原(PCT)水平与急性淋巴细胞白血病(ALL)患儿预后的关系。
方法 收集2019年6月至2021年11月在本院就诊的ALL患儿68例纳入观察组，同时期收取非恶性血液病患儿36例作为对照组。
统计两组患者血清IL-6、PCT水平，并分析观察组患者血清IL-6、PCT水平与临床特征及与预后的关系。
结果 观察组IL-6、PCT水平高于对照组，P <0.05。
血清IL-6、PCT水平与性别、年龄、免疫表型无关(P >0.05)，与Risk分层、白细胞计数、血红蛋白、血小板计数、原始细胞比例有关(P <0.05)。
IL-6高表达组PFS短于低表达组(χ2=32.588，P <0.001)，PCT高表达组PFS短于低表达组(χ2=66.004，P <0.001)。
结论 ALL患儿IL-6、PCT水平均出现明显上升的现象，且IL-6、PCT水平可能与ALL进展有关，通过检测IL-6、PCT水平可预测患者短期预后。
【关键词】急性淋巴细胞白血病；白细胞介素-6；降钙素原；预后【中图分类号】R733.71【文献标识码】A DOI:10.3969/j.issn.1009-3257.2023.05.038Relationship between Serum Interleukin-6 and Procalcitonin Levels and Prognosis in Children with Acute Lymphoblastic Leukemia SHANG Nan *, TIE Feng, LIU boratory Department of Kaifeng Children's Hospital, Kaifeng 475000, Henan Province, ChinaAbstract: Objective T o investigate the relationship between serum interleukin-6 (IL-6) and Procalcitonin (PCT) levels and the prognosis of children with Acute Lymphoblastic Leukemia (ALL). Methods A total of 68 children with ALL who were admitted to our hospital from June 2019 to November 2021 were included in the observation group, and 36 children with non-malignant hematologic diseases were included in the control group during the same period. The levels of serum IL-6 and PCT in the two groups were statistically analyzed, and the relationship between the levels of serum IL-6 and PCT in the observation group and clinical characteristics and prognosis was analyzed. Result s The levels of IL-6 and PCT in the observation group were higher than those in the control group (P <0.05). Serum IL-6 and PCT levels were not correlated with gender , age and immunophenotype (P >0.05), but were correlated with Risk stratification, white blood cell count, hemoglobin, platelet count and proportion of primitive cells (P <0.05). The PFS of the high expression group of IL-6 was shorter than that of the low expression group (χ2=32.588, P <0.001), and the PFS of the high expression group of PCT was shorter than that of the low expression group (χ2=66.004, P <0.001). C onclusions The levels of IL-6 and PCT are significantly increased in children with ALL, and the levels of IL-6 and PCT may be related to the progression of ALL. The detection of IL-6 and PCT levels can predict the short-term prognosis of patients.Keywords: Acute Lymphoblastic Leukemia; Interleukin-6; Procalcitonin; The Prognosis 急性淋巴细胞白血病(acute lymphoblastic leukemia，ALL)为常见恶性血液类肿瘤疾病之一[1]。
