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1、HLA不全相合非血缘脐血移植治疗无丙种球蛋白血症一例报告2005年7月第26卷第7期ChinJHematol,July200,:!HLA不全相合非血缘脐血移植治疗无丙种球蛋白血症一例报告万鼎铭刘长凤王桂菊孙慧孙玲姜中兴郭万里秦玲刘少君【摘要】目的探讨HLA不全相合非血缘脐血移植(CBT)治疗x连锁无丙种球蛋白血症(Bru—ton病)的疗效;观察其造血及免疫重建过程.方法为1例男性l4岁Bruton病患儿行HLA1个位点不相合的非血缘CBT.预处理方案为Bu/Cy(白消安/环磷酰胺)加抗CD3单抗.移植物
2、抗宿主病(GVHD)预防采用环孢菌素A+霉酚酸酯+甲氨蝶呤方案.移植有核细胞数0.42×10/kg,其中CD34细胞0.35×10./kg.结果移植后30天(+30天)中性粒细胞数0.5×10/L,BPC20×10/L,显示造血重建.+45天性染色体检测46,XX:46,XY为4:1.+100天性染色体均为46,XX;受者血型由AB型转为O型(供者型);IgG从1.1g/L升高到3.5g/L;成熟B细胞在外周血中从0上升到占0.05,显示免疫重建.随访360d,患者血常规,骨髓象正常,lgG13.5g/
3、L及成熟B细胞在外周血中占0.10,均为正常水平,未发生急,慢性GVHD,显示造血功能,免疫系统稳定重建.结论本例为首例非血缘CBT治疗Bruton病获得成功;HLA不全相合非血缘CBT治疗非恶性血液病安全,有效.【关键词l造血干细胞移植;丙种球蛋白缺乏血症;胎血SuccessfultreatmentofagammaglIobulinemiabyHLA-mismatchedunrelatedcordbloodstemcelltransplantationThefirstcasereportWANDing
4、—ming.LIUChang-feng.WANGGuiju,SUNHui.sUNLing,JlANGZhong—xing,GUOW帆一li,QINLing.LlUShao-jun.DepartmentofHematology.T}FirstAffiliatedHospitalofZhengzhouUniversity,Zhengzhou450052,China【Abstract】ObjectiveToevaluatecordbloodstemcelltransplantation(CBT)inthetr
5、eatmentofX—linkedagammaglobulinemia,andobservetheCOUleesofthehematopoieticandimmunereconstitution.MethodsA14一year—oldma
6、epatientwithagammaglobulinemiareceivedCBTfromal/6HLA.mismatchedunrelatedcordblood.TheconditioningregimenwasBu/Cy/anti.CD3antibody.CsAw
7、asgiventogetherwithMMFandMTXforprophylaxisofGVHD.Thepatientreceived0.42×10.nucleatedcells/kg.containing0.35×10.CD34cells/kg.ResultsTherecipientshowedhematopoieticreconstitutiononday30Dost.transplantationWhenANCwas0.5×10/LandBPC20×10/L.Sexchromosomeanalys
8、isshowedengraft.ment(donor46,XX/recipient46,XY:4:1)onday45.Therecipient'SbloodgroupchangedfromABtoO,IgGfrom1.1g/Lto3.5g/L,sexchromosomefrom46,XYtofull46,XX,andmatureBcellsinperiph—eralbloodfrom0to5%onday100,indicatingimmunereconstitution.Atthelastfollow—
9、upof360davs.thepatientwithoutacuteorchronicGVHDshowednormalhemogramandmyelogram.IgG13.5g/Land10%matureBcellsinperipheralblood,indicatingthehematopoiesisandimmunepersistentreconstitution.NoacuteorchronicGVHDwasdeveloped.Con
