小儿“非高危型”急性淋巴细胞白血病降低强度的规范化治疗

目的 了解“非高危型”急性淋巴细胞性白血病(AL)患儿降低治疗强度的可行性.方法 选取2007年1月至2012年2月期间应用改良Dutch ALLⅥ方案治疗35例“非高危型”ALL患儿为治疗组,选取同期应用改良IC-2002-AL方案治疗的“标危组”ALL患儿112例为对照组,比较两组患儿的诱导缓解率、3年无事件生存率、感染病死率、复发率指标和病人的住院治疗费用.结果 35例治疗组患儿诱导缓解率100％;3年无事件生存率85.71％;感染病死率2.86％;复发率11.43％;无失访病例;总治疗失败率14.29％;每位患儿平均住院次数8.60±2.40次;平均每次住院费用4 486.78±2 358.42元.112例对照组患儿诱导缓解率100％,3年无事件生存率60.71％;感染病死率7.14％;复发率7.14％;失访率25.00％;总治疗失败率39.29％;每位患儿平均住院次数9.80±3.90次;平均每次住院费用6 137.93±3 597.23元.两组患儿复发率差异无统计学差异(x2=0.65,P ＞0.05);3年无事件生存率,感染病死率和总治疗失败率差异均有统计学意义(x2值分别为7.50、4.22、5.78,均P＜0.05);两组患儿平均每次治疗费用差异无统计学意义(t=1.83,P＞0.05);平均住院次数差异有统计学意义(t=2.09,P＜0.05).结论 改良Dutch ALLⅥ方案治疗“非高危型”急性淋巴细胞白血病初步结果安全可行,疗效满意,费用低廉.     

Is acute lymphoblastic leukemia in children virus-related?

The sharp peak in incidence of acute lymphoblastic leukemia at ages 2 and 3 years strongly suggests the effect of an agent, whether viral or not, to which either exposure occurs only in the earliest months of life or to which immunity develops very rapidly. Suspected clusters of childhood leukemia in the neighborhoods of two British nuclear reprocessing facilities led Leo Kinlen to postulate that large-scale immigration into areas that had previously been remote and isolated offers opportunities for spread of viral infections to which most urban populations become immune at a very early age: leukemia may be a rare manifestation of infection by one or more of such viruses. He and his group have presented evidence in support of this hypothesis. Lack of increase in childhood leukemia in the contexts of the massive evacuation of mothers and children from British cities during the Second World War, and of the considerable immigration into previously isolated islands of Greece during the last several decades, indicates that some large movements of children have occurred without providing the circumstances postulated by Kinlen. The marked inverse association of leukemia risk with birth order, noted almost 30 years ago, remains unexplained and deserves to be recalled when considering the possibility of viral involvement in the etiology of acute lymphoblastic leukemia of children.     

儿童急性淋巴细胞白血病化疗后并发菌血症的影响因素分析

目的分析儿童急性淋巴细胞白血病(ALL)化疗后并发菌血症的影响因素。方法采用回顾性分析的方法,在无锡市儿童医院2013年8月-2017年1月接受化疗的全部ALL患儿中随机抽取454例样本为研究对象,记录患儿性别、年龄、病情危重分级、病原菌检出情况、病原菌菌株分布情况,进行影响因素的单因素和多因素分析。结果 454例ALL患儿菌血症发生率为20.48%,化疗后共发生菌血症118次,男性占比高于女性,主要集中于1～10岁;高危(HR)患儿并发菌血症占比最高,且主要集中在诱导缓解与巩固治疗阶段,呼吸道是其主要感染部位;共检出病原菌41类,121株,其中革兰阳性菌检出株数最多;经单因素与Logistic回归分析结果显示,HR、长时间使用抗菌药物、中性粒细胞缺乏、中性粒细胞缺乏持续时间长、中心静脉置管、多种抗菌药物联合使用、耐药性高等可能是ALL患儿化疗后并发菌血症的影响因素(P<0.05)。结论儿童ALL化疗后常并发以呼吸道感染为主的菌血症,疾病的发生可能与化疗期间长时间中性粒细胞缺乏、中心静脉置管、多种抗菌药物使用、耐药性等因素有关,临床应密切关注ALL患儿化疗期间中性粒细胞表达变化情况,尽量减少抗菌药物的使用,减少中心静脉置管,以降低菌血症发生风险,提高化疗有效性与安全性,改善患儿预后。     

Body composition in children in remission from acute lymphoblastic leukemia

BACKGROUND: Changes in body composition are commonly reported in pediatric survivors of acute lymphoblastic leukemia (ALL). However, the effect of ALL and of its treatment on body composition in children in remission from ALL has not been fully examined with the use of a reference method. OBJECTIVES: We aimed to determine the body composition and composition of fat-free mass (FFM) in children in remission from ALL. We also aimed to compare the effects that prednisolone and dexamethasone had on the body composition of an ALL survivor population. DESIGN: This cross-sectional study measured height, weight, body volume, total body water, and bone mineral content in 24 children in remission from ALL and 24 age-matched, healthy control subjects. Body composition and FFM composition were evaluated by using the 4-component model. RESULTS: The mean body mass index and fat mass index were significantly (P = 0.05 for both) higher in the ALL survivors than in age-matched control subjects. The composition of the FFM in the 2 treatment groups was not observed to differ significantly. Examination of the composition of FFM made it evident that children in remission from ALL had both significantly greater hydration (P = 0.001) and lower density (P = 0.0001) of FFM than did the control children. CONCLUSIONS: Children in remission from ALL may develop excess body fat. To measure body composition accurately in an ALL population, the high hydration and low density of FFM in this population should be taken into consideration.     

小儿急性淋巴细胞白血病长期存活61例分析

目的 对61例存活5年以上的小儿急性淋巴细胞白血病(ALL)患儿进行分析,探讨影响其长期存活的因素.方法 对这61例ALL患儿通过骨髓形态分型、免疫分型、染色体核型分析(MIC)、临床分型和微量残留病(MRD)检测,以及通过不同时期3组化疗方案及4种髓外白血病防治方法比较,了解其对疗效及预后的影响.结果 61例5年以上存活ALL患儿与176例5年以下存活患儿比较,二者染色体差异有显著意义(P=0.007),临床分型、骨髓形态分型及免疫分型比较差异无显著意义(P＞0.05).61例5年以上存活的ALL患儿中,不同时期3组治疗方案无病存活率比较,VCP(长春新碱、环磷酰胺、强的松)组与VDLP(长春新碱、柔红霉素、L-门冬酰胺酶、强的松)组间差异有显著意义(P=0.010).采用60Co头颅照射为主的治疗方案,3例发生中枢神经系统白血病(CNSL),采用大剂量氨甲碟呤(HDMTX)防治组,无CNSL发生,5例发生睾丸白血病(TL)的患儿均系60Co头颅照射组,HDMTX组无TL发生(P=0.000).61例5年以上存活ALL患儿中,25例已存活10年以上,现仍然存活,另36例存活5至10年者中,有5例死于骨髓复发,这两者骨髓复发的差异无显著意义(χ2=3.78,P=0.072).纳入本研究的所有ALL患儿中,26例MRD检测呈阳性,其中14例化疗停止时已转为阴性,13例仍长期存活;12例停止化疗后MRD呈弱阳性者,其中7例长期存活.结论 系统规范化疗,是ALL患儿长期存活的关键,骨髓复发、髓外白血病是影响存活的重要因素.MRD转阴,可以作为患儿停止化疗的重要参考指标.     

Survival of children diagnosed with acute lymphoblastic leukemia in 1977-82 in Connecticut

There was no evidence for an increase in survival among all all cases (N = 104) diagnosed between 1977-82 with acute lymphoblastic leukemia (ALL) at age less than 15 years and reported to the population-based Connecticut Tumor Registry (CTR). Hazard rates were consistently higher in males vs females during the second year after diagnosis, especially in males diagnosed after 1977-78. Registry data on prognostic factors, including white blood cell (WBC) count at diagnosis and Down's syndrome (DS), and initial treatment were used to interpret survival data by sex and year of diagnosis. A statistically significant sex difference in 4-year survival among cases diagnosed in 1979-80 was related to an unusually high survival rate (i.e. 100%) in females and the occurrence of two male DS cases. Further studies are needed on sex-specific survival rates among ALL cases diagnosed after 1977-78 in population-based cancer registries, which could be improved by more complete collection of data on immunophenotype and validation of data on relapses.     

Therapeutic results with apl 93 protocol in acute promyelocytic leukemia (34 cases)

BACKGROUND: Acute promyelocytic leukaemia (APL) account for approximately 10% to 15% of all AML in most reports. Clinical features includes the presence in 80% to 90% of patients of a severe hemorrhagic syndrome, a specific balanced translocation between chromosomes 15 and 17 with a fusion of a large pert of the retinoic acid receptor a gene (RARa) on chromosome 17 to a part of the promyelocytic leukaemia (PML) gene on chromosome 15.More than 75% of patients (under 65 years of age) can be cured, with the application of a combination of anthracyclines and all-trans retinoic acid (ATRA), followed by maintenance therapy. AIM: of the study was to assess of the therapeutic management of APL 93 protocol in acute promyelocytic leukemia. METHODS: We present here the results of a retrospective study concerning 34 patients with APL included between 1998 and 2004 in the APL 93 protocol : 20 in group B and 14 in group C. CR was 82 %. RESULTS: Failure is only due to toxic death (18%) Event free survival at 4 years is 63,47% with relapse rate at 14.25%. Overall survival at 4 years is 69,72%. Our results are acceptable and can be improved with reduction of failure due to toxic death, probably with omission of cytarabine from induction and consolidation adapted by the Spanish PETHEMA Group.     

Treatment of infants with acute diarrhea in Hungary

In the treatment of infants with acute diarrhoea complicated by mild to moderate dehydration, there is a great importance of rapid rehydration over 3-4 hours with oral rehydration solution containing 60 mmol/l sodium and then the rapid reintroduction of normal diet or breast feeding which should be continued all the time if possible. The pharmacological treatment of diarrhoea is not justified. In the present study which was part of a European multicentre survey using questionnaires the authors analysed how the recommendation of European Society of Pediatric Gastroenterology, Hepatology and Nutrition are followed in Hungary. It was evaluated 131 returned questionnaires, 80 of primary care and 51 of hospital-based pediatricians. The overwhelming majority of pediatricians (92%) recommended the oral rehydration at the beginning of treatment. The four fifth of them use the ORS with recommended composition. Only 31% of them use rapid oral rehydration over 3-4 hours. Merely 10% of them suggest the early reintroduction of normal feeding after the oral rehydration, while more than half of the respondent pediatricians (52%) think that temporarily the use of lactose free formula is justified. It is a very favourable fact that 84% of doctors recommend the continuation of breast feeding. Some kind of drugs for the treatment of diarrhoea is suggested by 15% of repliers. On the base of results it is obvious that the guidelines for the treatment of acute diarrhoea in infancy is complied only partially in Hungary and the reintroduction of normal feeding is delayed.     

强烈化疗治疗儿童急性淋巴白血病的临床分析

目的总结分析强烈化疗治疗小儿急性淋巴细胞白血病的临床疗效.方法 121例患儿采用(VDLP)方案诱导缓解,缓解后用CAT、HDMTX、EA、VDLP方案早期巩固强化治疗,用MTX、6-MP维持治疗,用MA、EA、VDLP方案定期加强治疗,用HDMTX联合三联鞘注对庇护所白血病进行预防.结果完全缓解率(CR)95.8%,标危ALL为97.2%,高危ALL为92%.5 a无事件生存(EFS)率为(70.2±6.2)%.标危ALL为(70.2±5.0)%,高危ALL为(55.3±8.9)%.结论采用多种非交叉耐药性抗肿瘤药物交替联合强烈化疗,庇护所预防治疗及完善的辅助治疗措施是减少白血病复发,提高EFS率的关键.     

急性淋巴细胞白血病患儿诱导缓解期医院感染的危险因素分析

目的 了解急性淋巴细胞白血病(ALL)患儿诱导缓解期医院感染的危险因素.方法 对医院儿科血液组 2005年1月-2011年8月住院治疗的374例初发ALL患儿进行回顾性调查研究,对发生医院感染病例进行单因素检验和多用素的非条件logistic回归分析.结果 374例初发ALL患儿发生医院感染161例,感染率为43.0％,多因素分析得出差异有统计学意义的危险因素分别为:年龄、体重、病房床位数、血红蛋白量、中性粒细胞数.结论 白血病患儿诱导缓解期因化疗及住院时间较长,易于发生医院感染,床位数是医院感染的危险因素,年龄、体重、血红蛋白、中性粒细胞数是医院感染的保护因素.     

左旋门冬酰胺酶治疗急性淋巴细胞白血病患儿的护理研究

目的探讨应用左旋门冬酰胺酶(L-Asp)治疗急性淋巴细胞白血病患儿的不良反应及护理对策。方法总结分析18例急性淋巴细胞白血病(ALL)患儿应用L-Asp的不良反应,并针对性落实相应的护理措施。结果不良反应发生率为65%,其中低蛋白血症5例,凝血障碍8例,肝功能异常7例,使用L-Asp前谷丙转氨酶(ALT)平均45 U/L,用后平均128 U/L。血象异常16例,用药前后差异有统计学意义。结论用药前后加强监测及病情观察,及时对症处理,能缓解及减轻患儿用药后的不良反应,促进疾病康复,提高生存质量。     

儿童急性淋巴细胞白血病和急性粒细胞白血病发病初期微量元素及营养相关蛋白的变化

目的 探讨儿童急性淋巴细胞白血病和急性粒细胞白血病发病初期某些微量元素及营养相关蛋白的变化。方法应用原子吸收光谱法、散射比浊法、干化学法和化学发光法等,对73例急性淋巴细胞白血病和I26例急性粒细胞白血病发病初期的患儿血清铜、锌、铁、镁、磷、钙、铜蓝蛋白、铁蛋白、转铁蛋白、乳酸脱氧酶、总蛋白、白蛋白、血红蛋自含量和红细胞数量等指标进行检测,并与30名正常儿童相比较,运用t检验分析两个发病组和正常对照组之间这些指标的变化。结果发病组与对照组相比,除锌和磷含量的差异无统计学意义外,其余指标的差异均有统计学意义（P〈0．05）,发病组的铜、镁、铁、铁蛋白、铜蓝蛋白和乳酸脱氯酶含量显著高于对照组（P〈0．05）,而钙、转铁蛋白、总蛋白、白蛋门、血红蛋门含晕和红细胞数量显著低于对照组（P〈0．05）。急性淋巴细胞白血病与急性粒细胞白血病相比,在铁及铜锌代谢的相关指标方面的差异有统计学意义（P〈0．05）。结论儿童急性淋巴细胞白血病和急性粒细胞白血病发病初期,血清中一些微量元素与营养相关蛋白含量与正常状态相比,发生紊乱,失去平衡。     

Significant association of acute lymphoblastic leukemia with HLA-Cw7

Frequencies of all defined HLA-A, -B, -C (-DR) antigens were determined in 142 (59) Germans suffering from acute lymphoblastic leukemia (ALL) with differentiation of immunologically defined or age-related subgroups of the disease. A highly significant rise of the HLA-C locus antigen Cw7 was found in ALL patients, particularly those over the age of 11 in comparison with local German and Caucasian controls of the Ninth Int. Histocompatibility Workshop (WS). Only slight differences of HLA-Cw7 frequencies were observed within the four immunologically defined ALL subtypes of all or age-related patient groups. HLA-A, -B, or -DR antigens, as well as HLA-ABC three-locus haplotypes were similarly distributed in patients and their local or Caucasian controls. The results indicate HLA-linked genetic factors conferring susceptibility to ALL in adults, particularly those presenting with B-, T-, and Null-ALL.