ph样急性淋巴细胞白血病的研究进展

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1、授予单位代码10089学号或申请号20153130中国图书分类号R733.71HebeiMedicalUniversity硕士学位论文专业学位Ph样急性淋巴细胞白血病的研究进展研究生：高婷婷导师：罗建民教授专业：内科学二级学院：河北医科大学第二医院2018年3月目录中文摘要··············································································1英文摘要·····················································

2、·························2英文缩写··············································································3综述Ph样急性淋巴细胞白血病的研究进展································5致谢····················································································27个人简历····················

3、··························································28中文摘要Ph样急性淋巴细胞白血病的研究进展摘要Ph样急性淋巴细胞白血病是前体B淋巴细胞白血病的一种高危亚型，具有侵袭性高，预后不良的特点，在儿童和成人的发病过程中具有特征的基因改变，没有明确的诊断标准。目前该病诊断主要基于基因学方面，特点为BCR/ABL融合基因为阴性，但基因表达谱却与Ph表达阳性的急性淋巴细胞白血病相似。该病多发于男性和唐氏综合症患者，且该病的发病率与年龄密切相关，其中儿童约占12%，青少年（16-20岁

4、）约占21%，成人（40岁以上）占20%~24%，而21岁至39岁的年轻人达到高峰占27%，因此在成人中高发并且趋于年轻化。该疾病与微小残留（Minimalresidualdisease,MRD）密切相关，严格监测MRD水平对指导治疗该疾病及预后风险评估具有重要意义，目前一些针对基因突变的靶向药物（Tyrosinekinaseinhibitors,TKIs）的研究应用为该病提供了方向及希望。本文旨在对Ph样急性淋巴细胞白血病的研究进展做一综述。关键词：Ph样急性淋巴细胞白血病,酪氨酸激酶抑制剂,靶向药,微小残留,诊断1英文摘要Theprog

5、ressofthestudyonPh-likeacutelymphoblasticleukemiaABSTRACTPh-likeacutelymphoblasticleukemiaisahigh-risksubtypeofBcellprecursorALL,whichrepresenthighaggressiveandleadtopoorprognosis.Ithasacharacteristicgenemutationinchildrenandadults.Thereisnodefinitediagnosticstandard,Itisc

6、urrentlymainlybasedongeneexpressionanalysis.Itischaracterizedbyagene-expressionprofilesimilartoBCR-ABL,withoutBCR-ABLfusiongene.ItoccursmoreofteninmaleindividualsandpatientswithDownsyndrome.Itisassociatedwithage,approximately12%inchildren,21%inadolescents(16-20yearsofage),

7、and20%to24%inadultsolderthan40years,withapeak(27%)inyoungadults21to39yearsold.Itisassociatedwithminimalresidualdisease(MRD).StrictmonitoringofMRDlevelisofgreatsignificanceforguidingthetreatmentofthediseaseandprognosisriskassessment.Tyrosinekinaseinhibitors（TKIs）providether

8、oadandhopeforthisdisease.ThisarticleprovidesanoverviewofthePh-likeacutelymphoblasticleuke