Objectives To analyze the long-term therapeutic outcome of patients with acute promyelocytic leukemia(APL)in children.Methods All 17 APL patients were treated with all-trans retinoic acid(ATRA)as induction therapy until achieved complete remission(CR),together with chemotherapy or combined with arsenic induction and chemotherapy;followed by 3-5 courses of consolidation chemotherapy until PML-RARα fusion gene negative were proved.Then they received ATRA,As2O3,6-MP,MTX maintenance therapy.Total courses was 2 years.All patients were examined for bone marrow morphology,karyotype and PML-RARα fusion gene before,every three months after chemotherapy,and every three to six months after treatment was completed.Karyotype and PML-RARα fusion gene were analyzed by R-banding,and RT-PCR respectively.Results Among all the 17 patients,16(94%)patients achieved CR,12(70%)patients achieved continuous CR(CCR),The mean follow-up was 35(7-122)months.Four(4)patients relapsed(25%).Two of them showed continuous positive PML-RARα fusion gene and the other 2 patients transformed from negative into positive.Conclusions Most APL patients treated with serial therapy of combined ATRA and arsenic induction and chemotherapy can achieve a higher CCR,regularly cytogenetic detections can be regarded as an important marker for prognosis of APL.

Objective To detect the argyrophilic proteins in nucleolar organizer regions(Ag-NORs) that express rDNA and rRNA proliferation of T lymphocytes before chemotherapy and after complete remission(CR) in children with primary acute leukemia(AL).Methods The argyrophilic granules area of NOR/nuclear area(I.S%) of T lymphocytes was detected by image analysis system in peripheral blood of 42 patients before chemotherapy and after CR and 30 normal children.Results I.S% in the patients before chemotherapy(5.06%±1.36%) were significantly lower than those in the healthy donors(7.51%±1.06%)(t=8.238 P0.001).I.S% in the patients after CR(7.17%±0.98%) were significantly higher than those before chemotherapy(t=7.073 P0.001).However,no significant difference was encountered in I.S% between the patients after CR and the normal children(t=1.403 P0.05).Conclusion These results suggest that decrease of Ag-NORs expresses the evidence for tumour induced suppression of immune function of T cells in children with AL prior to treatment.

目的通过对急性白血病( AL)患儿 T淋巴细胞( T细胞)表示 rDNA和 rRNA活性的嗜银蛋白的测定,了解患儿 T细胞的功能状态,并分析其与 T细胞亚群之间的关系.方法应用图象分析法测定 31例初发未化疗 AL患儿及 30例正常儿童(对照组)外周血 T细胞核仁组成区嗜银蛋白(银染区) /细胞核面积( I.S%),用流式细胞仪测定 T细胞亚群.结果 31例 AL患儿 I.S%值( 5.78%± 2.66%)较对照组( 7.51%± 1.06%)下降( P<0.01).T细胞亚群测定中, CD4+ ( 27.50%± 12.56%)较正常值( 35%± 2.55%)下降( P<0.05); CD8+ ( 26.11%± 12.35%)与正常值( 25%± 2.04%)比较差异无显著性( P>0.05); CD4+ /CD8+ ( 1.149± 0.51)较正常值( 2± 0.26)下降( P<0.001).结论在初发未化疗的 AL患儿中, T细胞嗜银蛋白含量下降,代表患儿 T细胞免疫功能的 CD4+和 CD4+ /CD8+值降低,显示患儿 T细胞活性的改变与 T细胞亚群的变化相符,患儿存在 T细胞免疫功能降低.

Objective To learn hematopoietic progenitor cells (HPC) proliferation though detection of BFU-E,CFU-E,CFU-GM and CFU-Meg by HPC culture of bone marrow (BM) from children with aplastic anemia (AA). Methods To detect clone formation unit (CFU) of BFU-E,CFU-E,CFU-GM and CFU-Meg at 7 d or 14 d after cellular culture from 47 children with AA. Results The mean values of four different colonies in 47 patients were significantly lower than those of the control BM. Meanwhile, the mean decrease of these colonies was positively correlated with the severity of the types of diseases. Among 47 patients, the CFU counts of BFU-E (36.17%),CFU-E (85.11%),CFU-GM(74.47%) and CFU-Meg(91.49%) were lower than those of the control. The CFU number of the patients with their BFU-E,CFU-GM and CFU-Meg lower than the donors were positively correlated with the severity of the disease and the decrease of their peripheral blood cells, but not with CFU-E. Conclusion A portion of children with AA have lower quantities of BFU-E,CFU-E,CFU-GM and CFU-Meg, and the quantitive decrease in these colonies is positively correlated with severity of the disease. The change of BFU-E, compared with the change of CFU-E, could represent erythroid proliferation of BM in children with AA.

目的分析31名血液病患儿服用乳果糖前后肠道菌群及SIgA含量.方法用培养方法检测肠道菌群,用单向琼脂免疫扩散法测定粪便中SIgA.结果服用乳果糖后,血液病患儿肠道内双歧杆菌数量增加,分泌SIgA水平上升.结论乳果糖刺激机体肠道内双歧杆菌生长以及免疫系统分泌SIgA,增加肠道局部免疫力.

目的探讨儿童急性淋巴细胞白血病(ALL)免疫表型与无病生存期(EFS)的关系.方法对48例初发未治疗的儿童ALL,用免疫酶标法测其骨髓肿瘤细胞的免疫表型.治疗采用1993年北海会议制定的<小儿白血病诊疗建议>.结果 40例为My-BL,其首次诱导完全缓解(CR)率97.5%,化疗2年内EFS 82.5%,3年内EFS72.5%.4例My+BL,首次诱导CR率50%,化疗2年内EFS 25%.3例My-TL首次诱导均CR,但EFS与My+BL基本类似.结论目前儿童ALL的免疫表型测定在判断其疗效与预后方面有着重要作用.

用单克隆抗体(单抗)对白血病细胞作免疫表型分析,目前已成为辅助诊断白血病及鉴别诊断白血病类型的手段之一.我们在1994年1月～2000年1月对83例未经治疗的儿童急性白血病(AL)进行了免疫表型分析.

由中国癌症研究基金会主办,白血病*淋巴瘤杂志社和解放军总医院血液科联合承办的第三届全国难治性白血病学术研讨会定于2001年10月上旬在北京召开.此次会议被列为国家级继续教育项目,颁发一级学会论文证书及国家级继续教育学分.会议将邀请著名专家作专题报告. 征文内容:有关难治性白血病的病因、发病机制、实验研究、临床诊断标准与治疗、造血干细胞移植、护理及预后因素等. 征文要求: 1.征文为全文并附800字左右的文摘,内容包括:研究目的、方法、结果和结论; 2.征文请详细附有工作单位、作者姓名,邮编、联系地址与电话号码; 3.征文需打印或字迹清楚地书写(最好寄软盘)请自留底稿; 4.征文请于2001年6月30日前至山西省太原市职工新街3号<白血病*淋巴瘤>杂志编辑部,邮编:030013,请在信封上注明"会议征文”字样. 5.联系电话:0351(6450389),传真:0351-4424182. 6.E-mail:BXBZJBJB@263.net 

骨髓造血干细胞移植(BMT)是近年来各国应用较为广泛的医治血液系统疾病的方法.BMT可分为自体和异体造血干细胞移植两种,由于种种原因均开展得很局限.一般无血缘关系的人群中,HLA配型一致的机率是1～2万分之一,再加之对骨髓移植的偏见使多数人不愿接受HLA配型检测,因此,干细胞的来源成为干细胞移植的一大难题. 脐血干细胞(umbilical cord blood stem cell,UCBC),取自来源广泛的脐血,富含CD34+细胞.CD34+细胞是具多系分化潜能的造血前体细胞,可能与细胞与细胞间的粘附及造血相关基因的表达有关.随胎龄增长,CD34+细胞表达比例显著下降,妊娠13周时CD34+细胞占11.1%,足月时仅占1.0%.所需造血干细胞最低数量随供体与受体间HLA相容性、病人抗HLA敏感性及年龄等因素而变化.人脐血CD34+ Thy+Lin-细胞被当作最原始的干细胞,每104个低密度单核细胞(LDMNC)中有一个干细胞.脐血中还包括早期和定向造血祖细胞,是成人外周血10倍,含CFU-GM(粒单系集落形成单位)、BFU-E(红系爆式集落形成单位)、CFU-L(淋巴系集落形成单位)、CFU-Meg(巨核系集落形成单位)和CFU-Mix(混合系集落形成单位).BMT时重建体内造血所需造血干细胞数量尚未确定.成人同种异基因骨髓移植最少需CD34+细胞≥2×105/kg,而1份脐血通常含(20～50)×104个干细胞.粒单集落形成细胞(GM-CFCs)为(1～10)×103/kg,而脐血GM-CFCs在(2～2.5)×106.每例脐血采集量约50ml～200ml,就可移植成功1个50kg体重的成人,因此1份脐血对于1名一般儿童已经足够.

近年来我国小儿血液病的治愈率和生存率已有长足进步，如急性淋巴细胞白血病5年无病生存(DSF)率达75%，急性非淋巴细胞白血病DSF率也达40%［1］。为了了解血液病患儿在较长期患病和治疗过程中的智力与心理行为问题，我们对部分血液病患儿进行了调查，现报道如下。 对象与方法 一、对象病例组取2000年1月～6月因血液病住院患儿共57例，其中男性40例，女性17例；年龄6岁～11岁34例，～16岁23例，平均年龄10.79岁。患急淋(ALL)25例，急非淋(NALL)11例，慢粒2例，再障7例，霍奇金病4例，非霍奇金病6例，血友病1例，慢性血小板减少症1例。病程最长12年，最短1月，平均病程2年～3年。对照组随机取正常在校生59名，其中男性42例，女性17例；年龄6岁～11岁36例，～16岁23例，平均年龄10.21岁。与病例组具有可比性。

目的通过对前白蛋白(PA)的检测,了解其作为急性淋巴细胞白血病对肝浸润的意义.方法对22例治疗前患儿和完全缓解后的17例患儿,通过免疫比浊法测定PA,并检测其它肝功能指标,以及30例健康儿童对照组.结果 22例治疗前患儿,9例PA下降(与对照组相比P<0.01),3例白蛋白降低,1例谷丙转氨酶、6例谷草转氨酶升高,1例总胆红素上升,治疗前PA异常的例数高于其它肝功能指标异常的例数.其中完全缓解后的17例患儿,PA及其它肝功能指标则均在正常范围.结论通过PA的检测,发现治疗前急性淋巴细胞白血病肝浸润时,PA的变化远较其它肝功能指标的变化为明显.

Objective To evaluate the significanace of serum proalbumin (PA) in hepatic infiltration caused by primary leukemia of pediatric patients before chemotherapy. Methods Serum PA was measured in 33 cases before chemotherapy and in 24 of them after complete remission and in 30 normal children by immunologic nephelometry, other liver function tests were performed simultaneously. Results Before chemotherapy, among the thirty three cases, 11 of them had low PA, compared with that of normal controls, P 0.01, 3 had low serum albumin, another 3 had elevated ALT, 9 had elevated AST and one had increased total bilirubin. The case number of low PA was greater than than of other abnormal liver function tests. PA and other liver functions tests in 24 cases with complete remission all recovered to normal. Conclusion PA is more sensitive than other liver function tests in hepatic infiltration of primary leukemia of children before chemotherapy. (Shanghai Med J, 2000,23∶357 358)

例１男，第２胎第１产，孕３４周，因胎盘早剥行急诊剖腹产。生后８天发现肝脾肿大，血红蛋白分别为１７５～１０５ｇ／Ｌ，于第１６天入院。母孕２个月时有先兆流产，曾注射黄体酮。体检：早产儿貌，轻度黄染。肝肋下４．５ｃｍ，剑下４ｃｍ，质中，脾肋下１．５ｃｍ，质...

高危急性淋巴细胞性白血病(简称"急淋")缓解后易复发,耐药性强,治疗效果差,选用异基因骨髓移植(Allo-B3MT)不失为有效的治疗方法.为此我们对1例Ph1阴性复发型急淋采用Allo-BMT治疗,报告如下. 患者系女性,8岁零2个月,Ph1阳性,诊断为急淋.经841方案治疗2个月后骨髓缓解,10个月后复发.虽经积极化疗,最终不能完全缓解.移植前骨髓检查原始淋巴细胞+幼稚淋巴细胞占0.055,伴周围淋巴结肿大,提示急淋复发趋势,预后不佳.

ＰＭＬ－ＲＡＲα融合基因在急性早幼粒细胞白血病中的意义王耀莉，杜玲珍，戴勤勇，张影梅，黄秋花，陈赛娟急性早幼粒细胞白血病（ＡＰＬ）约９０％具有特异性的染色体易位ｔ（１５；１７）（ｑ２２；ｑ２１）。我们于１９９３年３月～１９９４年１月对４例ＡＰＬ患儿用...