非血缘供者骨髓移植成功治疗重型β地中海贫血亚洲首报2例

We report our experience in successful unrelated-donor bone marrow transplantation in 2 cases of beta-thalassemia major, which are approved to be the first 2 cases in Asia. The 2 children receiving transplantation of the bone marrow from unrelated donors were diagnosed as having beta-thalassemia major, whose gene mutation type was homozygous and double heterozygote. High-resolution HLA typing found a mismatch with 1 sub-locus in both cases, and red blood cell type was mismatched in 1 case. Pretransplant conditioning protocol contained busulfan (BU, 16 mg/kg x b.w.), cyclosphamide (CY, 200 mg/kg x b.w.), anti-thymocyte globulin (ATG, 90 mg/kg x b.w.) and fludarabine (25 mg x d-1 x m-2). To prevent graft-versus-host disease (GVHD) episodes, cyclosporine-A (Cs-A) and methotrexate (MTX) were administered. The 2 cases experienced condition resembling serum sickness during pretransplant conditioning. After bone marrow transplantation, 1 case had grade acute GVHD in the skin, another developed grade acute GVHD in both the skin and intestinal tract, but the condition was brought under control in both cases after proper treatment. The time for peripheral neutrophil granulocyte recovery to above 0.5 x 10(9)/L was 19 and 16 days postoperatively, respectively, and the time of WBC recovery to normal was 54 and 80 days postoperatively. Platelet recovery to over 50 x 10(9)/L occurred on postoperative days 61 and 90, and Hb recovered to above 100 g/L in both case on days 110 and 83 respectively. The time of final blood transfusion was 53 and 62 days postoperatively for the 2 patients. Gene mutation type in the 2 cases was switched to normal of the donors. After retrieval of relative literature, the 2 cases were approved as the first 2 successful unrelated-donor bone marrow transplantation cases in Asia. This will provide a new possibility of donor supply for hematopoietic stem cells transplantation in thalassemia.     

异基因脐带血干细胞移植治疗假肥大型肌营养不良症一例

目的　探讨脐带血干细胞移植对假肥大型肌营养不良症(DMD)治疗的可行性。方法对1例确诊为有家族史的11岁DMD患儿经HLA配型,在脐血库中寻找到1份有5个位点相合的干细胞,其细胞数为移植治疗用量的2 6倍。采用白消安、环磷酰胺和兔抗胸腺淋巴细胞球蛋白预处理后进行异基因脐带血干细胞移植治疗;术后给予环孢素A、泼尼松龙和骁悉预防移植物抗宿主反应,前列腺素E1预防肝静脉阻塞综合征,丽科伟预防巨细胞病毒感染,粒细胞刺激因子惠尔血和丙种球蛋白进行对症支持治疗,定期检测血清肌酸激酶(CK)、造血重建、血型转变和PCR STR。结果　移植后第12天外周血白细胞为0. 5×109 /L,第14天1. 0×1.09 /L,中性粒细胞数为0 .6×109 /L, 第37天停用粒细胞刺激因子,白细胞波动在3 .34×109 /L~12 .2×109 /L;第27天血小板>20×109 /L,血红蛋白于第24天上升至88g/L,停用红细胞输注。移植后第42天患儿的血型从移植前的A型转变为AB型(移植干细胞为B型);PCR -STR检测均呈混合嵌入,供者的比例逐渐上升至55% ~65%。第38天出现Ⅰ度移植物抗宿主病,CK从6000U/L降至600~2200U/L。移植后第42天的运动功能较移植前有所改善。结论　异基因脐带血干细胞移植后短期内可恢复造血重建并改善患儿的运动功能。     

HX—97方案对外周血造血干细胞动员,采集和移植后造血重建的 …

为提高外周血造血干细胞动员、采集、和移植后造血重建的效率,作者从１９９７年４月至１９９年６月;进行了２２例异基因或自体外周血造血干细胞移植,对外周血造血干细胞动员、采集和移植后造血重建方案（ＨＸ－９７方案）作了系统观察。ＨＸ－９７方案的主要内容是：①外周血造血干细胞动员,采用ｒｈＧ－ＣＳＦ３００μｇ／天,皮下注射,共６天,第６剂在干细胞采集有９０分钟用;②自体外周血造血干细胞动员采用大剂量化疗加造     

MMC方案预处理后异基因外周血造血干细胞移植治疗慢性粒细？…

为进一步探讨慢性粒细胞白血病的治疗，对３例ＣＭＬ患者采用ＨＬＡ相合同胞史妹异基因外周血干细胞移植治疗，预处理方案为马法兰（Ｍ）１７０ｍｇ／ｍ＾２＊１，甲环亚硝脲（Ｍ）４００ｍｇ／ｍ＾２＊１，环磷酰胺（Ｃ）６０ｍｇ／ｋｇ＊２。     

Allogeneic peripheral blood stem cell transplantation in the treatment

Objective To investigate the efficacy of allogeneic peripheral blood stem cell transplantation (PBSCT) in the treatment of severe aplastic anemia (SAA) and severe infection. Methods A patient with SAA and pseudomonas aeruginosa septicemia was treated with PBSCT from an HLA-identical sibling with cyclophosphamide (CY) and total body irradiation (TBI) for conditioning. The patient was infused with 20.3×108/kg mononuclear cells including 61.0×106/kg CD34+cells following the conditioning regimen. Results Twenty days after PBSCT, the absolute neutrophil count (ANC) of 1.0×109/L was achieved, with platelet count >50×109/L. The donor origin of engraftment was confirmed by polymerase chain reaction (PCR) analysis of short tandem repeats at the end of the first, sixth and twelfth month. The patient’s body temperature dropped to normal level when her ANC reached 0.5×109/L on day 10, and the bacterial culture of blood sample became negative subsequently. Symptoms and signs of acute or chronic graft versus host disease (GVHD) were not observed in 30 months after PBSCT. Conclusions Hematopoiesis was reconstituted shortly after PBSCT. The combination of CY and TBI and the infusion of sufficient peripheral blood stem cells may contribute to the successful engraftment. PBSCT may be considered as the first choice when hematopoietic stem cell transplantation is needed for SAA patients complicated with severe infection.     

粒细胞集落刺激因子动员的异基因骨髓与外周血干细胞混合移植治疗血液系统恶性疾病

目的　探讨粒细胞集落刺激因子 (G CSF)动员的异基因骨髓与外周血干细胞混合移植治疗血液系统疾病的可行性与疗效。方法　供者给予G CSF连续 4～ 6天皮下注射 ,注射后第 3天从髂后上嵴抽取G CSF动员的骨髓血 ,第 4天开始取外周血干细胞。并立即输给受者——— 14例血液系统恶性疾病患者 (恶性肿瘤 10例 ,重症再生障碍性贫血 4例 )接受了G CSF动员的异基因骨髓与外周血干细胞混合移植 ,分析其植活率、植活速度以及急性、慢性移植物抗宿主病 (aGVHD ,cGVHD)的发生率。结果　 14例患者均获得异体植活 ,白细胞数恢复至≥ 1× 10 9/L ,血小板≥ 2 0× 10 9/L的中位时间分别为移植后的 14d(12～ 18d)和 13d (11～ 6 5d)。有 4例发生了Ⅰ度aGVHD ,3例发生了Ⅱ度aGVHD ,但无一例重度aGVHD发生。在可评估的 9例患者中有 3例出现了广泛的cGVHD。有 2例患者因白血病复发而死亡 ,另 1例因植活不良 ,再次输注供者外周血干细胞 (PBSC)后出现IV度aGVHD死亡 ,其余 11例中位随访时间 4 5 5d(84～ 715d) ,均无病生存。结论　G CSF动员的异基因骨髓与外周血干细胞混合移植可以用于血液系统恶性疾病的治疗 ,能保证持久、稳定的植活 ,植活速度快 ,急、慢性GVHD的发生率与异基因骨髓移植相比并没有增加。     

异基因造血干细胞移植后血小板植入失败行供者外周血造血干细胞输注治疗

目的对异基因造血干细胞移植（allo—HSCT）后血小板植入失败行供者外周血造血干细胞输注（GPBSCI）治疗的安全性、有效性进行初步评估。方法采用回顾性分析方法,针对2003年4月—2006年6月于北京大学血液病研究所行allo—HSCT的15例患者行16例次GPBSCI治疗的临床资料进行分析。结果15例患者中位年龄为33岁（14～48岁）,男性9例,女性6例,均为急慢性白血病患者,接受人类白细胞抗原（HLA）配型相合或不相合骨髓或骨髓联合外周血干细胞移植,回输骨髓单个核细胞（MNC）计数平均为（4．21±1．91）×10^8／kg[（1．50～7．46）×10^8／kg],回输外周血干细胞MNC计数平均为（3．27±1．40）×10^8／kg[（1．13—5．90）×10^8／kg],CD34^＋计数平均为（2．13±1．69）×10^6／kg[（0．24～5．67）×10^6／kg]。所有患者获得粒细胞植入,8例因原发性血小板植入失败,余8例因继发性血小板植入失败而实施GPBSCI治疗。回输中位时间为移植后＋113d（43～384d）。回输MNC计数平均为（3．09±1．54）×10^8／ks[（1．35～5．99）×10^8／kg]。仅1例患者在回输后出现急性移植物抗宿主病（aGVHD）表现。3例显著有效,6例有效,1例进步,6例无效。有效率为56．3％。其中移植后100d内进行2次回输疗效更好。结论对于allo—HSCT患者血小板植入失败,采取供者外周血造血干细胞2次回输,相关不良反应小,对促进受者造血恢复能够发挥一定的疗效。     

高白细胞值对血红蛋白测定的影响及纠正

目的　探讨高白细胞 (WBC)值对血红蛋白 (Hb)测定的影响及其纠正。方法　制备 4 8个不同浓度梯度的 WBC悬液 ;在全自动血细胞分析仪的预稀释模式下进行测定 ,观察去 WBC前后 Hb测定值的变化 ,建立校正Hb测定值的直线回归方程并应用回归方程对 1 4例高 WBC值全血标本进行可行性验证。结果　高 WBC值 (≥4 0 .0× 1 0 9/L )将使 Hb测定值明显升高 (P<0 .0 0 1 ) ;去 WBC前后 Hb测定值的差值与 WBC值的对数呈直线相关(r=0 .95 2 6 ) ,其回归方程为 Y(Hb差值 ) =- 2 5 .0 9+2 1 .89× lg[WBC(× 1 0 9/L ) ];应用回归方程计算高 WBC值全血标本去 WBC前后 Hb测定值理论差值 ,与实测差值比较二者无显著差异 (P>0 .0 5 )。结论　高 WBC的存在将使 Hb测定值假性增高 ,通过线性回归方程校正可得到 Hb的真实值     

非清髓异基因造血干细胞移植联合伊马替尼治疗慢性粒细胞白血病的临床研究

目的　探讨非清髓造血干细胞移植联合伊马替尼(格列卫、STI571)在治疗慢性粒细胞白血病(CML)中的作用。方法　10例CML患者中3例为慢性期,4例为加速期,3例为急变期。移植前、后口服格列卫(400～1500mg/d)治疗,预处理方案为福达拉宾,环磷酰胺和阿糖胞苷联合抗胸腺细胞球蛋白或CD3单抗。供者HLA配型4例完全相合,2例1个位点不相合,1例2个位点不相合的同胞,1例3个位点不相合的同胞及2例半匹配的母亲供者。干细胞来源为重组人类粒细胞集落刺激因子(rhG- CSF,格拉诺赛特)动员的外周血造血干细胞(PBSC),移植物抗宿主病(GVHD)的预防以环孢菌素A和霉酚酸酯(骁悉)为主,部分病例加用甲氨蝶呤、CD3单抗及CD25单抗(塞尼派)。结果　10例患者均获得不同程度的嵌合状态,3例获得完全嵌合(>95%),7例获得44%～95%的混合嵌合。7例混合嵌合状态的患者经调整免疫抑制剂、供者淋巴细胞/PBSC输注,格列卫治疗,6例患者在移植后1 5～10个月转变为完全嵌合。移植后中性粒细胞>0. 5×109/L所需天数为16d(10～21d);血小板大于20×109/L所需天数为10d(4～15d)。移植期间1例患者移植后45d因肠道感染,颅内出血死亡。另1例患者移植后27d因多脏器衰竭死亡。8例患者随访7～23个月,6例发生Ⅰ～Ⅱ度GVHD,2例发生Ⅲ～Ⅳ度GVHD,除1例因慢性GVHD死     

人类白细胞抗原不相合造血干细胞移植治疗白血病的临床研究

目的 :探讨应用人类白细胞抗原 (humanleukocyteantigen ,HLA)不相合供体造血干细胞移植治疗白血病。方法 :总结我所于 2 0 0 0年 7月至 2 0 0 1年 12月进行的 7例HLA不相合造血干细胞移植 ,其中包括 3例慢性髓性白血病、3例急性非淋巴细胞性白血病、1例急性淋巴细胞白血病。干细胞来源 :6例为外周血干细胞 ,1例为骨髓干细胞。预处理方案为改良马利兰 (busulfan ,BU) /环磷酰胺 (cyclophosphamide,CY)或BU/CY +抗胸腺细胞球蛋白 (antithymocyteglobulin ,ATG)。预防急性移植物抗宿主病 (graftversushostdisease,GVHD)采用环孢霉素A及短疗程氨甲喋呤 ,5例患者加用霉酚酸酯 (骁悉 )。结果 :1例为骨髓造血干细胞 ,采集骨髓单个核细胞数 3 .4 1× 10 8kg-1,6例为粒细胞集落刺激因子 (granlocytecolony stimulatingfactor,G CSF)动员后外周血干细胞 ,平均接受8.4 6× 10 8kg-1(4.3 0× 10 8～ 15 .3 5× 10 8kg-1)供者外周血单个核细胞 ,平均 + 13天 (+ 11～ + 16天 )中性粒细胞(absoluteneutrophilcount,ANC)大于 0 .5× 10 9L-1。平均 + 16天 (+ 11～ + 2 3天 )血小板大于 2 0 .0× 10 9L-1。发生急性Ⅰ～Ⅱ度GVHD 3例 (42 .9% ) ,无 1例严重的急性GVHD ,发生慢性广泛性GVHD 2例 (2 8.6% )。中位随访时间 1     

异基因外周血干细胞移植治疗高危白血病

目的　探讨异基因外周血干细胞移植(allo-PBSCT)治疗高危白血病的疗效。方法　25例高危白血病患者,中位年龄34（5.5～52）岁,接受了HLA配型相合同胞供者外周血干细胞移植,其中急性白血病（AL）15例,（第一次完全缓解1例,为Ph染色体阳性,第二次及以上完全缓解期7例,复发7例,包括2例异基因骨髓移植(BMT)后复发;慢性粒细胞性白血病（CML）4例;（第2次慢性期、加速期、急变期、BMT后复发各1例）;骨髓增生异常综合征(MDS)6例,移植物抗宿主病（GVHD）预防方案采用经典环孢霉素（CsA）加氨甲蝶呤（MTX）。结果　所有患者均植活,中性粒细胞数恢复至≥0.5×109/L和血小板数≥20×109/L的中位时间分别为移植后14(10～18) d和11（7～45） d。发生II度及以上急性GVHD13例,包括1例III度GVHD,未发生IV度急性GVHD。23例可评估患者中16例(70%)诊断慢性GVHD。移植相关死亡率为16%,复发6例,4例经回输供者淋巴细胞获得再次缓解。19例患者无病存活,中位随访时间为304（94～1 963） d。2年总生存率、无病生存率及复发率分别为64%、58%及25%。结论　allo-PBSCT可治疗高危白血病患者,降低移植后复发率,延长无病生存。对有高危因素的血液系统恶性肿瘤患者,选择PBSCT替代骨髓移植更有优势。     

小鼠单倍体相合外周血移植模型的建立

目的建立单倍体相合外周血移植小鼠模型,探讨移植后造血及免疫重建特性。方法经大剂量全身照射预处理BDF1小鼠后,经尾静脉输注新生第一天DBA/2小鼠的外周全血,移植后动态观察受体鼠的造血重建及急性移植物抗宿主病(GVHD)发生情况,并用流式双染色法进一步检测粒系、淋巴系造血细胞的长期植入水平。结果移植后小鼠的白细胞、血小板、血红蛋白分别在第18、21、18天明显回升,其中白细胞和血小板分别于第29天和36天恢复正常,而血红蛋白至第50天仍为部分恢复。受体鼠90%长期存活,且不发生明显的GVHD。移植后第50天流式细胞仪检测供鼠来源的造血细胞占总有核细胞的88.81%,其中含供者来源的粒细胞33.92%,T细胞16.83%。结论单份新生小鼠外周血含有足够的造血干细胞,能重建单倍体相合小鼠的造血及免疫系统而不引起明显的GVHD,并可形成稳定的以供者细胞为主的嵌合体,其中粒细胞为完全嵌合。     

脐血造血干细胞移植治疗小儿慢性粒细胞白血病

目的 :研究脐血造血干细胞移植治疗小儿慢性粒细胞白血病的疗效及特点。方法 :给一例 12岁男性慢性粒细胞白血病慢性期的患儿移植HLA配型基本相合的同胞脐血。预处理选用BU CY方案 (马利兰 4mg·kg- 1 × 4d ,环磷酰胺 6 0mg·kg- 1 × 2d)。预防移植物抗宿主病 (GVHD)采用环孢霉素A(CsA)。移植有核细胞数为 2 0× 10 7·kg- 1 。结果 :移植后 2 2d(+2 2d)微卫星位点分析显示移植物植入成功 ,+2 5d骨髓显示造血开始重建 ,+42d中性粒细胞 >0 .5× 10 9·L- 1 ,+91dBCR ABL融合基因表达转阴 ,且受者血型由O型转为供者血型B型。随访 15 0d ,未发生急、慢性GVHD。结论 :脐血造血干细胞移植对小儿慢性粒细胞白血病有根治性治疗作用 ,它具有GVHD发生率低、程度较轻、重建造血慢等特点。     

Autogeneic peripheral blood hemopoietic stem cell transplantation for chronic myeloid leukemia with imatinib mesylate-induced negative Philadelphia chromosome]

OBJECTIVE: To study the possibility of curing chronic myeloid leukemia with autogeneic hemopoietic stem cell transplantation in patients with negative Philadelphia (Ph) chromosome induced by imatinib mesylate (STI 571) treatment. METHODS: Two patients with chronic myeloid leukemia in chronic phase, who had 90% Ph chromosome-positive cells and bcr/abl fusion gene-positive cells as shown by interphase fluorescence in situ hybridization (I-FISH), failed to respond favorably to interferon-alpha therapy in the treatment courses of 7 and 8 months, respectively. Treatment with STI 571 at a daily dose of 300 to 400 mg for 5 months to 8 months was subsequently implemented, after which the Ph chromosome and bcr/abl fusion genes became normal in detection for 3 times. Peripheral blood haemopoietic stem cell mobilization was then initiated by intravenous injection of cytarabine (2.0 g/d) for 3 days, etoposide (0.2 g/d) for 3 d and cyclophosphamide (1.0 g/d) for one day. When the white blood cell was below 1.0x10(9)/L, the G-CSF (300 microg/d) was administered subcutaneously for 5 or 6 d, and the peripheral blood mononuclear cells were collected by CS3000 Plus blood cell separator. The percentage of bcr/abl fusion gene-positive cells among CD34(9) cells enriched by MiniMAC ranged from 11% to 14%. After 3 or 4 weeks, the patients received total body irradiation at 9 Gy given in 2 fractions, with intravenous injection of cyclophosphamide (60 mg/kg daily) and etoposide (300 mg/d) for 2 d. On the day of transplantation, the collected mononuclear cells were 4.17x10(8)/kg and 3.9x10(8)/kg, with CD34(+)/ cells reaching 4.89x10(6)/kg.b.w and 4.89x10(6)/kg. CsA was also used since day -1 to day +13 of the transplantation for prevention of graft-versus-host disease. G-CSF was administrated daily at the dose of 300 microg subcutaneously from day +3 to +12. RESULTS: After the transplantation, the absolute neutrophil count (ANC) took a mean of 11 d to exceed 0.5x10(9)/L in these two patients, and 19 and 21 d, respectively, were needed for the platelet count to exceed 20x10(9)/L. The two patients showed cytogenetic relapse at 120 and 300 d after the transplantation, respectively. CONCLUSION: Autogeneic peripheral blood stem cells transplantation after Ph chromosome is negative in patients with chronic myeloid leukemia, who receive STI 571 treatment, may also relapse, and more radical elimination of Ph chromosome-positive cells is needed.     

由Hb H病和重型β地中海贫血引起的一个罕见中间型地中海贫血病例及其产前诊断应用

目的 分析1例中间型地中海贫血(地贫)患者的基因型与表型的关系,探索进行重症β地贫复合血红蛋白H病这类复杂基因型组合方式的产前诊断临床实践.方法 表型检测采用标准的血液学分析技术测量红细胞参数和血红蛋白组分.采用反向点杂交技术诊断β地贫基因突变,采用缺口聚合酶链反应技术检测α地贫缺失突变.通过基于家系调查的表型与基因型分析探讨该病例的发病机制.利用羊水细胞DNA对该家系1例地贫高风险胎儿进行产前基因诊断.结果 先证者诊断为β0/β 地贫(βCD17A＞T/βIVS2-654C＞T)双重杂合子复合血红蛋白H病(--SEA/-α4.2)的偏重的中间型地贫患儿,其父母分别为β地贫杂合子合并轻型α地贫和β地贫杂合子合并静止型α地贫个体.在此基础上,我们对该家庭的地贫高风险胎儿进行了产前基因诊断,结果显示胎儿的基因型为重症β地贫合并静止型α地贫,通过遗传咨询实施了中止妊娠.结论 首次报道罕见中间型地贫案例,为国内外产前诊断提供可借鉴的临床经验.     

Application of autologous peripheral blood stem cell transplantation in children with malignant tumor

Objective To investigate if low dose total body irradiation (TBI, 6.0-9.0 Gy) combined with intensified chemotherapy followed by autologous peripheral blood stem cell transplantation results in better survival in children with refractory leukemia or solid tumors. Methods Twenty-one children with malignant tumors were included in this study.There w ere 14 males and 7 females aged 3.5-12 years.Underlying disease included high -risk acute lymphoblastic leukemia (ALL, CR(1) in 3 children and CR(2) in 5 ch ildren), acute myeloblastic leukemia (AML, 9 children), non-Hodgkin’s lymphoma stage Ⅳ ( 2 children), and neuroblastoma stage Ⅳ (2 children).The peripheral hematopoi etic stem cells were collected six to eleven months after complete response, mob ilized with high dose chemotherapy alone or combined with GM-CSF or G-CSF.Th e conditioning regimen consisted of chemotherapy with two to three combinations of the following drugs: cyclophosphamide, arabinosylcytosine, McNU, etopside, an d Idarubicin on the basis ofTBI (6.0-9.0 Gy).A mean of (1.8±0.5) ×10(8)/kg autologous mononuclear cells were transplanted.The patients were followed up after transplantation. Results Severe bone marrow suppression occurred in all patients around day +7.Peripher al white blood cell count decreased to 0 in all patients at day +4.8±2.9, and platelet count decreased to less than 20×10(9)/L at day +9.0±2.6.Succ essful engraftment was achieved in 21 patients, but four died of infection at da y +17, +20, +31 and +67, respectively.Recovery of white blood cell (WBC) to 10×10(9)/L, absolute neutrophil count to 0.5×10(9)/L, platelet count to 20 ×10(9)/L occurred on 21±12, 26±13, and 27±10 days, respectively.During the follow up period, three patients relapsed at +5 months, +1.5 years, and +2 yea rs 10 months, respectively.One patient died of intracranial hemorrhage at +8 m onths.Thirteen patients had event-free survival for 2-12 years, with a mean o f 6.7±3.4 years.Conclusion Our preliminary data suggest that myeloablative therapy with low dose TBI (6.0 -9.0 Gy) combined with intensified chemotherapy followed by autologous periphe ral blood stem cell transplantation might be associated with favorable results i n children with refractory leukemia or solid tumors.     

阿糖胞苷联合粒细胞集落刺激因子动员自体外周血干细胞效果的研究

目的　观察阿糖胞苷 (Ara C)联合重组人粒细胞集落刺激因子 (rhG CSF)对恶性淋巴瘤患者自体外周血造血干细胞 (APBSC)的动员效果 ,并寻找Ara C合适的给药剂量。方法　按照入组的先后顺序 ,将患者分成两组 ,A组Ara C的给药剂量为 6g/m2 静滴 (分 2次 ,间隔 2 4h) ,B组Ara C的给药剂量为 10g/m2 静滴 (分 4次 ,间隔 12h) ,白细胞 (WBC)降至最低点时开始皮下注射rhG CSF 30 0 μg·人 -1·d-1,直至采集结束前 1d ,白细胞恢复到 5 .0× 10 9/L以上时开始连日采集APBSC ,当累计采集的单个核细胞≥ 5× 10 8/kg或CD34+ 细胞≥ 2× 10 6/kg时停止采集。结果　 2 2例患者进入本研究 ,A、B两组各有 11例患者。Ara C给药后 ,患者外周血中白细胞和中性粒细胞绝对值 (ANC)的最低值 ,B组明显低于A组 ,出现的时间B组也明显晚于A组。A、B两组rhG CSF给药的开始时间和持续时间、APBSC采集的开始时间和持续时间均无显著差异 ,在APBSC采集时的循环血量、血流速和采集时间相同的情况下 ,APBSC的采集次数、每次采集的细胞数量和总量亦差异无显著意义 ,B组Ara C引起的某些毒副反应略重于A组 ,但两组间差异无显著意义。结论　Ara C联合rhG CSF是一种安全、高效的APBSC的动员方法 ,6g/m2 的Ara C即可得到满意的动员效果。     

Allogeneic peripheral blood stem cell transplantation for leukemia

Objective To observe engraftment kinetics, the incidence and severity of graft-versus-ho st disease (GVHD), and clinical outcome on 40 recipients undergoing allogeneic p eripheral blood stem cell transplantation (allo-PBSCT).Methods From June 1997 to May 1999, forty leukemia patients with a median age of 35 year s underwent allo-PBSCT. PBSC were mobilized with G-CSF at a dose of 5 μg/kg s.c. every 12 hours for 5 days. A median of 7.7 (2.0-16.8)×10 (6) CD34(+) cells/kg was infused into the recipients. Busulfan-cyclophosphamid e (BU-CY) was used as the conditioning regimen. All patients received cyclospo rine A and either methotrexate (n=34) or methylprednisolone (n=6) for GVHD p rophylaxis. Results Engraftment of neutrophils and platelets was achieved at a median of 13 days (9 -28 days) and 12 days (7-60 days) respectively. Patients receiving ≥4×10 (6) CD34(+) cells/kg or given G-CSF post transplant had significantl y accelerated neutrophil and platelet engraftment. Acute GVHD occurred in 17 of 40 patients (42.5%), with grade Ⅱ-Ⅳ acute GVHD in 10 patients (25%). Chron ic GVHD developed in 21 (9 extensive, 12 limited) out of 30 evaluable patients ( 21/30, 70%) with a median follow up of 380 days (180-900 days). Transplan t related mortality was 17.5% and the relapse rate was 10%. The probability of leukemia free survival at 3 years was 72.5%.Conclusion Allo-PBSCT can provide rapid hematopoietic reconstitution without an increased incidence of acute GVHD, but may be associated with a high risk of chronic GVHD .     

中国人群成人静脉血细胞分析参考范围调查

目的　建立中国正常成人静脉血细胞分析参数的参考范围。方法　在全国 14个城市(哈尔滨、长春、北京、天津、兰州、西安、南京、苏州、成都、武汉、重庆、福州、昆明、广州 )的 14家医院 ,使用不同型号的血液分析仪 ,检测正常人群 (男性 92 7人 ,女性 82 2人 ,共 174 9人 )静脉血白细胞计数、红细胞计数、血红蛋白浓度、红细胞平均血红蛋白含量 (MCH )、红细胞平均血红蛋白浓度(MCHC)、红细胞平均体积 (MCV)和红细胞压积 (HCT)。 14家医院均在同一个月内完成调查。研究期间 ,各实验室均进行严格的室内质量控制 ,保证结果的准确性和稳定性。结果　各项参数的参考范围 (均值 )如下 :白细胞计数男性为 (3 97～ 9 15 )× 10 9/L(6 0 3× 10 9/L) ,女性为 (3 6 9～ 9 16 )× 10 9/L (5 89× 10 9/L) ;红细胞计数男性为 (4 0 9～ 5 74 )× 10 12 /L(4 91× 10 12 /L) ,女性为 (3 6 8～ 5 13)×10 12 /L(4 2 6× 10 12 /L) ;血红蛋白浓度男性为 131～ 172 (15 1) g/L ,女性为 113～ 15 1(12 9) g/L ;MCH男性为 2 7 8～ 33 8(30 8)pg ,女性为 2 6 9～ 33 3(30 2 ) pg ;MCHC男性为 32 0～ 35 5 (335 ) g/L ,女性为 32 2～ 36 2 (32 5 ) g/L ;MCV男性为 83 9～ 99 1(91 2 )fl,女性为 82 6～ 99 1(91 3)     

双份无关供者脐血移植治疗成人体重急性白血病

目的 探索非亲缘性双份脐血移植（CBT）治疗成人体重急性白血病的可行性及并发症的防治。方法 对3例成人体重（〉50kg）急性白血病患者（1例ALL-NR，2例ANLL．CRl）进行双份无关供者脐血移植，预处理方案采用白消安／环磷酰胺（BU／Cy）方案加抗胸腺细胞球蛋白（ATG），移植物抗宿主病（GVHD）的预防用环胞菌素A（CsA）、甲氨蝶呤（MTX）及霉酚酸酯（MMF）±赛尼哌（Zenapax）；肝静脉闭塞病（VOD）的预防用低分子右旋糖酐及肝素。结果 例1、例2中性粒细胞绝对值（ANC）〉0．5×10^9／L的时间分别为＋17d、＋16d。血小板〉50×10^9／L的时间为＋40d、＋37d；DNA指纹图分别在＋18d、＋21d外周血VNTR检测显示为供者1型；例1＋33d骨髓象完全缓解；＋120d出现复发。例2染色体核型为46，XY；于＋55d血型转为A型。目前已无病存活50个月。例3于＋27d回输自体外周血干细胞，恢复自身造血，目前无病生存46个月。结论 对于成人体重受者接受HLA配型部分相合的2份脐血移植在临床上是可行的。