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爱心驰援华容,星城创想50万元助力灾后重建
2025年01月09日,诗社每次举行都是重大风雅活动,都是一次文化大地震。但诗社开了多次,还从来没有哪次堪比芦雪广。
《金瓶双艳》高清完整版在线观看-免费电影 - 果树剧集网《杨思敏金瓶梅》完结手机在线播放 - 电影武侠剧全集...
是众人爱心接力
“这个行业兴起的背后,不仅仅是一件衣服,更多的是传统文化的复兴、历史文脉的传承。希望通过汉服产业的繁荣发展,让更多人通过汉服了解到文化的魅力,并不断传承下去。”糖果说。美国消费者新闻与商业频道报道称,经济学家对中国的长期增长前景持乐观态度。报道援引高盛集团的分析称,由于调整优化防控措施并允许更大规模的人口流动,2023年中国经济增长将达到5.2%。随着旅行恢复正常,中国经济增长将带动中国周边地区经济增长。
>>>迟补颈驳耻辞办耻补锄丑补苍驳蹿补苍驳蝉丑补颈尘补辞,诲补辞锄丑颈蝉耻濒颈蝉耻辩颈诲颈蝉补苍,锄丑颈丑耻颈肠丑别苍驳蝉丑颈箩颈补苍蝉丑别测颈肠丑别苍驳飞别颈驳辞苍驳箩颈补苍“测颈诲补颈测颈濒耻”诲别锄丑辞苍驳测补辞锄丑颈肠丑别苍驳,迟辞苍驳驳耻辞箩颈补辩颈补苍驳蝉丑耻锄颈箩颈苍驳箩颈驳耻辞箩颈肠丑补苍苍别苍驳丑别锄耻辞,测辞耻濒颈测耻箩颈补苍驳诲补蝉丑耻箩耻、测耻苍箩颈蝉耻补苍、谤别苍驳辞苍驳锄丑颈苍别苍驳诲别苍驳虫颈苍箩颈蝉丑耻测颈苍驳测辞苍驳诲补辞锄丑颈丑耻颈肠丑别苍驳蝉丑颈箩颈补苍蝉丑别锄丑辞苍驳,迟耻颈诲辞苍驳肠丑耻补苍迟辞苍驳箩颈肠丑耻蝉丑别蝉丑颈丑别蝉丑耻锄颈箩颈肠丑耻蝉丑别蝉丑颈谤辞苍驳丑别蹿补锄丑补苍,肠丑辞苍驳蹿别苍濒颈测辞苍驳肠丑别苍驳蝉丑颈诲补蝉丑耻箩耻濒补颈测辞耻丑耻补锄颈测耻补苍辫别颈锄丑颈丑别飞补苍蝉丑补苍肠丑别苍驳蝉丑颈驳辞苍驳苍别苍驳,尘补苍锄耻肠丑别苍驳蝉丑颈箩耻尘颈苍谤颈测颈锄别苍驳肠丑补苍驳诲别蝉丑耻锄颈丑耻补、锄丑颈苍别苍驳丑耻补、濒惫蝉别丑耻补虫耻辩颈耻,驳别苍驳丑补辞诲颈飞别颈谤别苍尘别苍蝉丑别苍驳肠丑补苍丑别蝉丑别苍驳丑耻辞蹿耻飞耻。
光(骋耻补苍驳)源(驰耻补苍)的(顿别)位(奥别颈)置(窜丑颈)不(叠耻)高(骋补辞),光(骋耻补苍驳)线(齿颈补苍)的(顿别)强(蚕颈补苍驳)度(顿耻)也(驰别)不(叠耻)高(骋补辞),最(窜耻颈)适(厂丑颈)合(贬别)拍(笔补颈)摄(厂丑别)“剪(闯颈补苍)影(驰颈苍驳)”了(尝颈补辞)。
jingliliao2021niandegeweishuzengchang,2022nianyinxingyekuobiaozengsukaishixiufu,zongzichanyuzongfuzhaideguimozengsushuangshuangzhandaoliao10%yishang,huigui2020deshuiping。ertonglinbamuxibaolinbaliuzhenliaoguifan(2019 nianban)2019-10-28 16:25·zhongliujie2022yi、gaishulinbamuxibaolinbaliu(lymphoblastic lymphoma, LBL)shiyizuqiyuanyubuchengshuqianti T huo B linbaxibaodeexingzhongliu,yuezhanertongfeihuoqijinlinbaliu(Non Hodgkin Lymphoma, NHL)de 35%~40%,shiertong NHL zuichangjiandebinglileixingzhiyi。youyu LBL yujixinglinbaxibaobaixuebing(acute lymphocytic leukemia, ALL)juyouxiangsidelinchuangheshiyanshitezheng,zhexietezhengbaokuoxibaoxingtaixue、mianyibiaoxing、jiyinxing、xibaoyichuanxueyijilinchuangbiaoxianheyuhou,yincidangqian WHO fenleijiangerzhegongtongguiyuqiantilinbaxibaozhongliu,mingmingweiqianti T/B linbamuxibaobaixuebing/linbaliu(T/B-ALL/LBL)。danglinchuangyizhongliuxingbingzaoqibingerwugusuijiwaizhouxuejinrunhuogusuizhongzhongliuxinglinbamuxibaoxiaoyu25%shizhenwei LBL;dangyougusuihewaizhouxueshoulei,gusuizhonglinbamuxibaodayu 25%shi,zezhenwei ALL。anzhaomianyibiaoxingfenwei T xibaoxing(yuezhan70%~80%)he B xibaoxing(yuezhan 20%~30%)。jinnianlai,tongguocaiyongyuweixianduxiangguandeleisizhiliao ALL defanganhualiao,shiliaoxiaodedaoxianzhutigao,yixiezhiliaozhongxin 5 nianwushijianshengcunlv(Event-free survival,EFS)yidadao 75%~90%。er、shiyongfanweiyijuhuanerlinchuangtedian,jingzhongwuhuojian、xiongfushuihuogusuixibaoxingtaixue、mianyibiaoxing、xibaoyichuanxuehefenzishengwuxuejiancequezhen,bingjingzhishaoliangjiasanjijiadengyiyuanbinglizhuanjiahuizhenjieguoyizhi,an WHO 2016linbaxitongzhongliubinglizhenduanfenxingbiaozhunjinxingzhenduanhefenxingdeertongheqingshaonian LBL。san、zhenduan(yi)linchuangbiaoxian1.qianti T linbamuxibaolinbaliu(T-LBL):qian T xibaozhongliu85%~90%biaoxianwei LBL,shaoshubiaoxianwei ALL。haofayunianchanger,zhongweifabingnianling 9-12 sui。nanxingduojian,nannvzhibi(2.5~3):1。dianxingdelinchuangbiaoxianweiqianzongzuozhongwu,chuxianqingzhongbudengdeqidaoyapozhengzhuangrukesu、xiongmen、chuanxi、qicu、duanzuohuxideng,banxiongmoqinfankehebingxiongqiangjiye,jiazhonghuxikunnan;zongzuozhongwuyaposhiguankeyinqitunyankunnan;yaposhangqiangjingmaikezhijingmaihuiliushouzu、jingmianbuheshangzhishuizhong,ji"shangqiangjingmaizonghezheng(superior vena cava syndrome)";qinfanxinbao,kedaozhiexingdexinbaojiyehexinbaotiansai。linbajiebingbianyuezhan 70%,yijingbu、suogushangheyexiaduojian。bufenhuanzhehebingganpizhongda。yue 50%gusuishoulei;yue 15%zhongshushenjingxitongzhuanyi,naomobingbianduoyunaoshizhibingbian;xingxianshouleiyuezhan 5%。benbingwangwangjinzhanxunsu,90%yishangbinglijiuzhenshiyichuyulinchuangⅢ、Ⅳqi。2.qianti B linbamuxibaolinbaliu(B-LBL):yuqianti T xibaozhongliuxiangfan,qian B xibaogouchengdezhongliu 85%yishangbiaoxianwei ALL,zhiyou 10%~20%wei LBL。B-LBL fabingnianlingjiaoxiao,zhongweinianlingxiaoyu 6 sui。fabingwumingxianxingbietezheng。changjianbiaoxianweilinbajiezhongdajipifu、ruanzuzhi(youqishitoujingbu)、gu(changjianyugugu、zuogu、jizhugu)dengjiewaiqinfan,biaoxianweipifuduofaxinghongsejiejie,guneigulixingzhongkuai,yingxiangxuejianchashirongguxinghuoyinghuaxingbingbian;shaojiandebiaoxianyouzongzuo、xiongmoqinfan,neizangqinfanrushenzang、xiaohuadaojinrundeng。yeyifashenggusuihe CNS jinrun。(er)shiyanshijiancha1.binglijiancha:suoyoubingrenjunxutongguozhongliuzuzhihuogusuibinglihuojianquezhen。(1)binglixingtaixue:linbajiejiegoupohuai,zhongliuchangqinfanlinbajiebaomo、jiewaizhifanghexianweizuzhi。liuxibaochengmimanxingzhimidexiangduidanyixingjinrunshengchang,xibaozhongdengdaxiao,hezhibigao,heyuanxing、luanyuanxinghuoquxinghe,ransezhixirufenchen,herenchangbumingxian,baozhixishaodanran,hefenliexiangduojian。xibaoxuetezhengyu FAB fenxingzhongde L1 huo L2 xingyouzhilinbaxibaoxiangduiying。liuxibaojianhenshaoyouqitafanyingxingxibaochengfen,bufenbinglikejian"xingkongxianxiang"。(2)mianyibiaoxing:①linbamuxibaodebiaozhi:baokuo TdT、CD99、CD34、CD1a。LBL laiyuanyuqiantide T huo B linbaxibao,TdT beirenweishiqiantilinbaxibaodetezhengxingbiaozhi,xiongxianhuogusuiqian T、qian B xibaojunkechengxianyangxingbiaoda,wenxianbaodaoqiyangxinglvkeda 90%~95%。CD99 yebeirenweishiqianT xibaodelianghaobiaojiwu,dantabujinbiaodayurendexiongxianpizhixibaoyiji ALL/LBL xibaozhong,zai Ewing's rouliu(ES)、yuanshishenjingwaipeiyezhongliu(PNET)yijixiongxianliuzhongyejunyouyangxingbiaoda,yincizaijianbiezhenduanshangbixupeiyixiangyingdeqitayouxiaobiaozhilianheguancha。②T-LBL fashengyuxiongxianzuzhi,zhongliuxibaolaiyuanyuqianxiongxianxibao、xiongxianxibao,biaoda CD3、CD2、CD5、CD7,CD7 shiqiteyixingkangyuan。muqianrenwei T-LBL laiyuanyuxiongxianzhongfayuxiangduichengshudeqianti T xibao,er T-ALL jiaozhiyougengzaoqidefenhuajieduan。COG baogaoliao 180 li T-LBLdemianyibiaoxingtezhengyu T-ALL xiangsi,dan CD4/CD8 shuangyangxingdefashenglvmingxiangaoyuhouzhe,yezhichi T-LBL juyougengchengshumianyibiaoxing。③B-LBL zhongliuxibaolaiyuanyugusui,shuyuqianqian B、qian B、putongB jieduan,biaoda CD10、CD19、CD20、CD22、CD79a,qizhong CD79awei B xibaoteyixingkangyuan。Mayer dengbaodao 22%B-LBL cunzaimianyiqiudanbaiγqingliandequeshi,erzai B-ALL zhongdefashenglvjinwei 1%,yetishiliaoB-LBL laiyuanyujiao B-ALL shaochengshujieduandeqianti B xibao。④zaoqian T linbaxibaobaixuebing/linbaliu(ETP-ALL/LBL):qiyuanyuzuichufenhuajieduandexiongxianxibao,juyouxiang T xi、suixi、shutuzhuangxibao、NK xibaofenhuadeqianneng,mianyifenxingtedian:quefa CD1a、CD4、CD8 debiaoda;CD5 ruobiaodahuozhebubiaoda;zhishaoyouyigesuixihuoganxibaoxiangguandekangyuanbiaoda(CD13、CD33、CD117、CD11b、CD34、HLA-DRdeng);biaoda CD7、CD2、CD3 deng T xibaobiaozhi。yuezhan T-ALL/LBLde 10%~15%。benxingyuhoujiaocha。2.xibaoyichuanxue(Cytogenetics)hefenzishengwuxue(Molecular biology)benbingyuduozhongjiyinyichangyouguan,baokuokangyuanshoutijiyin、ransetiyichang、yiaijiyinshihuoyijiaijiyindejihuodeng。tongguoransetihexingfenxi、FISH fangfa、ronghejiyindingliang RT-PCR、IgH/TCR zhongpaijiance、xinyidaicexu(NGS)defangfajinxingxiangguanjiancha。95%yishang T-ALL/LBL you T xibaoshouti(T cell receptor,TCR)jiyinkelongxingzhongpai,daduoshu T-ALL biaoxianweifenhuachengshuguochengzhongshouxianzhongpaide TCRγσlianjiyin,er T-LBL zeduoweiαβlianjiyinzhongpai,yetishiliao T-ALL defenhuajieduanzaoyu T-LBL。dayue 20%T-ALL/LBL tongshiyou IgH jiyinzhongpai。juedaduoshu B-LBL you Ig-H jiyinzhongpai,yekeyou Ig-L jiyinzhongpai。50%~70%de T-ALL/LBL youransetihexingyichang。daduoshuhexingyichangyufeisuijixingduandianyouguan:changleiji TCRασ(14q11.2),β(7q35),γ(7q14-15),baokuocharutubian、queshiyijiransetiyiwei。yiweitongchangshiyuanaijiyinjihuobingyichangbiaoda,daozhiyuxibaozhouqidiaokonghexibaofenhuaxiangguandebajiyinjihuoerzhizhongliu。xianyizhiyuT-ALL/LBL fabingyouguandezhongyaoyuanaijiyinyou T xibaojixingbaixuebingjiyin 1(TAL-1)/SCL、HOX11、HOX11L2、LYL1、LMO1 he LMO2 deng。del(9p)queshidaozhideyiaijiyin CDKN2A dediushiyeshibenbingfashengdejizhizhiyi。muqianyizhijuyoujihuotubianleijibianmaqian T xibaofayudeguanjiandanbaideNOTCH1jiyindebingrenyuhoulianghao,fashenglvyuewei50%;eryue 10%debingrenjuyouranseti 6q zahexingqueshi(Loss of heterozygosity at chromosome 6q;LOH6q),qifufafengxianzenggao,yuhoubuliang。yue 60% B-ALL/LBL youtezhengxingyichuanxuegaibianqiejuyouzhongyaodelinchuangzhenduanheyuhoupanduanyiyi。yichuanxueshangtishiyuhoujiaohaodeyouchaoerbeiti(ransetishu>50)hexingji t(12;21)(p12;q22)yiweixingchengTEL/AML1 ronghejiyin;eryaerbeiti(ransetishuxiaoyu 45)、t(9;22)(q34;q11.2)xingcheng BCR/ABLl、 t( 4;11 )huo MLL/AF4 、 t(1;19)(q23;13.3)xingchengde(E2A-PBX1; TCF3/PBX1)yiji Phyangjiyinhuotubiandengyulinchuangliaoxiaobujia、yuhoubuliangxiangguan。3.xuechanggui:baixibaokezhengchanghuoqingdushenggao,keyoupinxue,duoweizhengxibaozhengsesuxingpinxue。danggusuishouleishi,keyoubaixibaozongshushenggaohuojiandi,waizhouxuechuxianyouzhixibao,kebanyoupinxuehe/huoxuexiaobanjiandi。4.xueshenghua:ganshengongneng、rusuantuoqingmei(LDH)、dianjiezhishibichaxiangmu。wangwangbiaoxianweiniaosuan、rusuantuoqingmeizenggao,zheliangxiangzhibiaoduijibinghuanjieqingkuangjiyuhouyoutishizuoyong。5.ningxuegongneng:baokuo PT、APTT、TT、FIB、D erjuti、FDP。benbingkezaochengningxuegongnengyichang,ningxuemeiyuanhexianweidanbaiyuanjianshao,congerdaozhiningxuemeiyuanshijianyanchanghechuxue。6.gusuijiancha:gusuishouleishi,gusuizhongyouzhilinbaxibaoyibanxiaoyu 25%,tongguoliushixibaoshu、xibaoyichuanxue、jiyinzhongpaidengjianchayouzhuyuzhenduanhefenxing。liushixibaoyijiancekeyifaxianyinxingdegusuishoulei。gusuihuojianduigusuiqinfandejianchulvgaoyugusuixingtaixue。7.naojiyejiancha:changguixingnaojiyechanggui、shenghua、shuaipianzhaozhongliuxibaoliaojieyouwuzhongshushenjingxitong(Central nervous system, CNS)deqinfan。liushixibaoyijiancezhongliuxibaomianyifenxingketigao CNS qinfanjianchulv。8.yingxiangxuejiancha:zhiliaoqianxuchangguijinxingquanshenyingxiangxuejianchayimingqueshouleibuwei,xiezhufenqi。X xianjiancha:gupian(yougu、guanjiezhongtongzhe);B chao:zhishaobaokuojingbulinbajie B chao、fubu B chaojibingzaobuweixiangguan B chao(zuozuidaliuzaoceliang);CT:zhishaobaokuoxiongfupen CT pingsao(ruozuo PET/CT,kebuzuochanggui CT jiancha,jinzuobingzaobuweijiancha);bingzaobuweixuxingzengqiang CT jiancha;huaiyizhongshushenjingxitongbingbiandexingtouluhe(huo)jisui MRI jiancha;youtiaojianzhejinkenengxingquanshen PET/CT jiancha。(san)ertong LBL dezhenduanbiaozhun1.zhenduanbiaozhunWHO 2016youguanlinbazaoxuezuzhizhongliufenleizhongguidingliaoLBLdezhenduanbiaozhun。chuyijuhuanzhelinchuangtedianwai,junxujingguoshouleizuzhihuojian,jinxingzuzhibinglixue、mianyibiaoxing、xibaoyichuanxuehefenzishengwuxuedejiancequezhen。linchuangfenqi:genjuquanshenyingxiangxue、naojiyejigusuidengjiancha,yiwanganzhaoSt Judefenqixitongquedingfenqi(jianfubiao1)。suizhuoyixuefazhan,ertongNHLxindebingliyaxingdezengjia,xibaoyichuanxue、fenzishengwuxuehemianyibiaoxingfangmiandejinbu,xindezhenduanfangfazaijianceweixiaokuosanbingzaohuoweixiaocanliubingzaodelinchuangyingyongyijiyingxiangxuefangmiandezhongyaojinzhan,zaiyuanyouSt Jude fenqixitongshangjinxingliaobuchonghegailiang,chanshengliaoxindefenqixitong:xiudingguojiertongNHLfenqixitong(IPNHLSS),jinyibumingquehebuchongliaomeiyiqidedingyi,bingjiangzuixindeyixuejinzhanxinxijiaruliaofenqixitong(jianfubiao2)。2.gusuiqinfandezhenduan:xuyaoxinglianggebuweigusuichuancihezhishaoyigebuweigusuihuojianjinxingfenxidingyigusuiqinfan。(1)gusuichuancixibaoxingtaixue:gusuiyouzhixibaohuolinbaliuxibao≥5%。(2)gusuimianyibiaoxingfangfa(mianyizuzhihuaxuehuoliushixibaoshufenxi:tezhilinbaliuxibaobaifenbi)yangxing。(3)gusuixibaoyichuanxuehuoFISHfenxiyangxing。(4)gusuiPCRfangfajiancefenzishengwuxueyangxing。(5)gusuihuojiankeyijiandaozhongliuxibaojinrun。3.zhongshushenjingxitongqinfandezhenduan:naojiyezhuangtaifenjizhuyaoyijulinchuangbiaoxian、yingxiangxuegaibian、naojiyejiancha。(1) CNS1:tongshifuheyixiasantiao:①naojiyezhongwuzhongliuxibao;②wuzhongshushenjingxitongyichangdelinchuangbiaoxian;③wuzhongshushenjingxitongyichangdeyingxiangxue(CT/MRI)yiju。(2) CNS2:fuheyixiarenheyitiao:①yaochuanwusunshang—naojiyebuhunxue(hongxibao:baixibao≤100:1)shi,naojiyebaixibaojishu≤5/μl,bingjiandaomingquedezhongliuxibao;②yaochuanyousunshang—naojiyehunxue(hongxibao:baixibao>100:1)shi,naojiyejiandaomingquedezhongliuxibao;③yaochuanyousunshangbingweixuexingnaojiye,bulunyouwuzhongliuxibao,ruchuzhenbaixibaoshu>50×109/L zeguiwei CNS2;④zhongshulinjinbuweideqinfanweifasheng CNS qinfandeweixianyinsu,weiliaojiaqiangyufangguiwei CNS2:toumianbupifu、ruanzuzhihuoguzuoqinfan,rulugu、bizuo、yankuangdeng(weitupoyingnaomo);jizhugupohuaihuozhuipangqinfanbubanshenjingxitongzhengzhuangde,wu CNS zhanwei、naojiyejianchazhengchang。(3)CNS3:fuheyixiarenheyitiaowei CNS qinfan:①naojiyebaixibaojishu>5/μl,bingyizhongliuxibaoweizhu,tongshihongxibao:baixibao≤100:1;huozhenaojiyebaixibaojishu>5/μl,qizhongzhongliuxibaosuozhanbiligaoyuwaizhouxueyouzhixibaobaifenbi;②lushenjingmabi,jishinaojiyezhongwuzhongliuxibao、lunao MRI/CT weifaxianzhanweixingbingbian;③CT/MRI kejiannao、jisuihuonaomo、jimobingbian。④jizhugupohuaihuozhuipangqinfanyinqipainiaoyichang、zhitihuodongzhangaidengshenjingxitongzhengzhuang。4.zuowanqinfandezhenduan:huanerbiaoxianweizuowandancehuoshuangcezhongda,zhidibianyinghuochengjiejiezhuangquefadanxinggan,touguangshiyanyinxing,B chaojianchakefaxianzuowanchengfeijunzhixingjinrunzao,chuzhenhuanerkebuyuhuojian。zaiquanshenhualiaohuanjiedehuanerchuxianzuowanzhongdazhe,yingjinxinghuojianyiquedingshifouzuowanfufa。(si)jianbiezhenduan1.jixingsuixibaixuebinghelixibaorouliu:xingtaixueshangchengsuixibaoyuLBL delinbamuxibaonanyiqubie。danchengsuixibaoxingtaijiaobuyizhi,xibaozhijiaoduo,baozhineikejianhongranbangzhuang Auer shixiaoti。mianyibiaoxing MPO、CD13、CD14、CD15 he CD64 chengyangxingfanying;TdT yiji T huo B linbaxibaobiaozhiweiyinxing。2.Burkitt's linbaliu:xingtaixueshangerzheyouxiangsizhichu,junweizhongdengdaxiaodedanxingxingzhongliuxibaogoucheng,dukechuxian"xingkongxianxiang"。danLBL oujianzaoxing"xingkongxianxiang",er Burkitt's linbaliu"xingkongxianxiang"changchangguanchuanyuzhenggeliuzuzhi。liuxibaobiaoda sIgM、CD10、EBER1/2 yuanweizajiaoyangxing。xibaoyichuanxuexianshiyoutezhengxing t(8;14)(q23;q21)huo c-myc jiyinzhongpai。3.feilinbazaoxuexitongxiaoxibaoxingexingzhongliu:zhuyaobaokuoyouwenshirouliu、yuanshishenjingwaipeiyezhongliu、shenjingmuxibaoliu、xiaoxibaoweifenhuaaideng。youqishifashengyulinbajiewaidexiaoyuanxibaozhongliu,youyuqimuxibaohuatezheng,qieertongyiweihaofanianlingzu,yincidang CD99(+),er LCA、CD3、CD20 junyinxingshiyiwuzhenweicileijibing。kexuanyongduozhongkangyuan,baokuoshenjingneifenmibiaojiwu(CgA、Syn)dengkezijianbie。4.xiongxianliu:yibanweiyuqianshangzongge,jishaofashengyuertongjiqingshaonian。congbinglishangliangzhejunbiaoxianweilinbaxibaomimanxingshengchang,TdT junchengyangxing。xiongxianliuliuzuzhichengfenyezhuang,jianmingxiandexianweibaomojicudadexianweijiange;sanzaidedifenzizhiliangjiaodanbai(CK)yangxingxibaoguanchuanyuzhenggeliuzuzhi。er LBL zhong,youyuliuxibaodejinrunxingshengchang,zaicancundexiongxianzuzhizhongkechuxianshaoliangzaoxingfenbude CK yangxingxibao。5.jitailiu:zonggeneikenengfashengliangxingheexingjitailiu,yeduofashengyuqianzongge,zhongliuneihanyougaomidudewuzhirugaihuaruangu、gu、chidengzuzhishi,X xianjianchachangkequedingzhenduan;xuezhongjiataidanbaizenggaozheduoweiexing。6.qitazonggezhongliu:rushenjingyuanxingzhongliu、xianweiliu、zhifangliu、linbaguanliudeng,junxutongguolinchuangbiaoxian、zhongliuzaizonggezhongdeweizhi、yingxiangxuetedianjibinglijianchajinxingjianbie。(wu)linchuangzhiliaofanyingpinggubiaozhun、pinggushijiandianjineirong1. pinggubiaozhun(1)liuzaohuanjieqingkuang①wanquanhuanjie(CR):tongguochatiheyingxiangxuejianchazhengshisuoyouliuzaoquanbuxiaoshi。CT xianshiyuanzhongdadelinbajiehezhongkuaiyisuoxiaozhizhengchangfanwei(zuidajingxian< 1.5 cm),geqiguanyingxiangxuekejiandaodesuoyouliuzaojunyixiaoshi。PET saomiaoyiwuyangxingfaxian。gusuitupian/huojianzhengchang;liushixibaoshujinxing MRD jianceweiyinxing。②weiquedingdewanquanhuanjie(Unconfirmed complete response):suiranbingzaozuidajing>1.5cm,danzongtishangbingzaosuoxiao>75%(xianghuchuizhiliangtiaozuidahengjing)。congyingxiangxueshangwufaquedingshifouweicanliudezhongliuxingbingzao,yingxingbinglihuojianqueding,eryoudebingzaoyoukenengbushiyushoushuhuojianqueding。③bufenhuanjie (PR):liuzaozuidajingxiansuoxiao≥ 50% ,meiyouxinfaliuzao。④wufanying(NR)(jibingwending):pingguxianshimeiyoudadao PR, danyemeiyouxinfaliuzao。⑤jibingjinzhan (PD):renheliuzaozengda≥ 25% huochuxianxinfaliuzaohuogusuifufa。(2)fenzishengwuxuehuanjie(MCR):jiancefenzishengwuxuebiaoji,lirutongguo PCR fangfajiancegusuironghejiyincongyangxingzhuanweiyinxing。(3)fenzishengwuxuefufa(huoyinzhuanyang):ruyoufenzishengwuxuebiaoji,zhiliaohouzhuanweiyinxingdejiyinhuoqitaronghejiyinzaicijianceweiyangxing。2.hualiaoqijianpinggushijiandian(1)qiangdesongyuzhiliao d8:pingguwaizhouxueyouzhixibaoshujizhuyaoliuzao;(2)VDLPfanganyoudaozhiliao d15jinzuogusuichangguiji MRDpinggu;(3)VDLP fanganyoudaozhiliao d33;(4)CAM2 fanganhualiaohou:chengweizhongqipinggu;(5)yanchiqianghuaⅠkaishiqian;(6)yanchiqianghuaⅡkaishiqian;(7)weichizhiliaoqijian:weichizhiliaokaishishi:baokuozhongjianweichijidaweichikaishiqian;weichizhiliaoqiansigexunhuanmei 8 zhoupingguyici(baokuogusuijianchajiliuzaopinggu),yihoumei 8 zhouxiaopingguyici(jiandanyingxiangxuejiancha,wuguchuanheyaochuan),mei 16 zhoudapinggu 1 ci(baokuonaojiyehegusuijiancha)。3.hualiaoguochengzhongpingguneirong(1)qiangdesongyuzhiliao d8:jinxingwaizhouxueyouzhixibaopinggujizhuyaoliuzaopinggu,yizuozhuyaoliuzaode B chaojianchaweizhu,zongzuozhanweikexingCT jiancha,bingxingzhongliujingxianceliang,zhongliuhuisuodayu 25%、waizhouxueyouzhixibaoxiaoyu 1000/mm3weiduiqiangdesongmingan;(2)VDLP fangan d15:yougusuiqinfandebingrenfuchagusuixingtai,bingtongguoliushixibaoyijiancegusui MRD;(3)qitashijiandianpinggu:baokuo①gusuihuanjieqingkuang:gusuixingtaiheliushixibaoyijiancegusui MRD;gusuironghejiyinyangxingzhexufuchagusuijiyinzhuanyin。②liuzaoqingkuang:liuzaobuweixiangguanyingxiangxuejiancha。si、linchuangweixiandufencengLBL weixiandufencengshangwutongyibiaozhun,yuanzeshangshizonghelinchuangfenqi、zhongliuxibaoyichuanxuetezhengyijizhiliaofanyingdengjiayiqueding。zaizhiliaozaoqixugenjuweixianyinsu,quedingweixiandufenzuweidiweizu、zhongweizu、gaoweizu。(yi)diweizuanzhaoxiudingdeguojiertong NHL fenqixitong,bujuyougaoweiyinsudeⅠ、Ⅱqibingren(cunzaizaoqizhongliuzifarongjiehuojudaliukuaideⅡqibingrenchuwai)。(er)zhongweizubujuyougaoweiyinsudeⅢ、Ⅳqibingren。(san)gaoweizuxutongguoliuzao、gusui、waizhouxuepingguliaojiezhiliaofanying。1.qiangdesongyuzhiliao d8,waizhouxueyouzhixibaodayu 1000/mm3。2.youdaozhiliao d15 gusuiyouzhixibao>25%。3.youdaozhiliao d33 zhongliucancun>25%huogusuiyouzhixibao>5%;gusui MRD≥10-2;naojiyezhongchixucunzaiyouzhixibao(zhisanciqiaozhuhounaojiyezhongrengyouzhongliuxibao)。qizhongrenyixiang。4.gongguzhiliaoqian(CAM2 hualiaohou)pinggurengyoucanliubingzaozhejinliangxinghuojian,rengweizhongliuzuzhi(ruguomeiyoutiaojianzuoercihuojiankeshitiaojianxing PET/CT xiezhujianbie);gusui MRD≥10-3。qizhongrenyixiang。5.juyoubuliangyichuanxuetezheng:t(9;22)huo BCR/ABL,t(4;11)huoMLL/AF4jiqitaMLLjiyinzhongpai、PhyangALLxiangguanjiyinruIKZF1、CRLF2、JAK2 deng。wu、zhiliao(yi)xitonghualiao1.hualiaoyuanze:jiyu LBL shengwuxuetexingleisiyu ALL,jinnianlaiguojishangcaiyongleisi ALL dehualiaofanganhouxianzhugaishanliaoyuhou,bijiaoguojijingdianfangan,yi BFM90 liaoxiaozuijia,benjianyitigongdezhiliaofanganshiyi BFM90 weijichudegailiangfangan,geyiyuankegenjugeziqingkuangxuanzeyingyong。yunxuduisuocaiyongdefanganjinxingweidiao,quyuzhongxinhuodadeyiliaozhongxinkezaiyuanzebubiandejichushangtichuhelixiugai。2.hualiaoqianzhunbei(1)quezhenjiancha:jinkenengzaizuirongyiqucaidebuweiqudebiaobenxunzhaozhongliuxibao,baokuo:gusui、xiongfushui、qianbiaolinbajie、pifukeyiliuzao、shenbulinbajiechuancihuohuojian、xiongfuqiangliuzaochuancihuoshoushuquhuojian。ranhouwanshanzhongliuxibaode MICM fenxing;wanchengzhongxinbinglihuizhen(zhishaoliangjiasanjiayiyuanbinglizhuanjiahuizhen)。(2)jianyixionggujizuogulianggebuweigusuichuancihuohuojian:yingxinggusuixibaoxingtaixue、liushixibaomianyibiaoxingfenxi、xibaoyichuanxuehejiyinjiancha。(3)changguijiancha:baokuoquanxuexibaofenxi、baixibaofenlei、ganshengongneng、xueqingrusuantuoqingmei、xiongfushuijiqitatiyedexibaoxuejianchadeng。(4)naojiyejiancha:xibaolixinfazhaoliuxibao、liushifangfajianceweiliangxibao。(5)yingxiangxuejianchajinxingfenqi:xingkeyilinbajiejifubu B chao、keyishouqinbuweideguzuopian、CT dengyingxiangxuejianchayibianliaojiebingbianfanwei。huaiyizhongshushenjingxitongbingbianshikexingnaojijisuicigongzhen(MRI)jiancha。youtiaojiandexing PET/CT jiancha。(6)hualiaoqianjiancha:liaojiezangqigongneng、mianyizhuangkuang、bingduganran、chuanranbingdengzhuangkuang。3.changguihualiao:yi BFM90 weijichudezhiliaofanganbaokuo VDLP+CAMyoudaohuanjiezhiliao、4 liaocheng HD-MTX(jiaandiezuo)huo 6 liaochenggaoweifangandehuanjiehougongguzhiliao、VDLD+CAM yanchiqianghuazhiliao、6-MP+MTXdeweichizhiliaodenghuanjie。butongyu ALL,LBL zhongⅠ、ⅡqihuanzhewuyanchiqianghuaⅡzhiliao,yanchiqianghuaⅠzhiliaohouzhijiejinruweichizhiliao。yuqitaleixing NHL butong,LBL zaidadaowanquanhuanjiehou,huanxujinxingweichizhiliao,qimudeshiyoudaozhongliuxibaofenhua、diaowang,dadaochediqingchucanliubingzaodemude。zongliaochengyuewei 24 geyue。zhiliaoguochengzhongxudingqijinxingpinggu,baokuogusuichanggui、gusui MRD dejiancejiliuzaodepinggu,liaojiehuanjiezhuangtai,jishidiaozhengzhiliaofangan。4.zhongshushenjingxitongjinrundeyufangjizhiliao:yuanzeshangquchuliaolunaofangliao(baokuo CNS3 bingren),yijiaqianghualiao、zengjiaqiaozhujiaqiang CNS qinfandeyufanghezhiliao。(1)CNS1 dezhiliao:suoyoubingrenjunjieshouyufangxingsanlianqiaozhuji 4liaochengdajiliang MTX hualiao,qizhong T-LBL 5 g/m2;B-LBL 3 g/m2。zhenggehualiaoguochengzhong,B-LBL zhishao IT 20 ci,T-LBL 22 ci。(2)CNS2 dezhiliao:zaiyoudaohuanjie VDLP fanganzhong(di 8、22tian)zengjia 2 cisanlianqiaozhu,jimeizhouyici,gong 5 ci。quanchenggong 22~24 ci。(3)CNS3 dezhiliao:zaiyoudaohuanjie VDLP fanganzhong(di 8、22tian)zengjia2cisanlianqiaozhu,jimeizhou1ci,gong5ci。yanchiqianghuaⅠ VDLD(di 1、15 tian)zengjia 2 ciqiaozhu。quanchenggong 26~30 ci。(4)weichizhiliaoqijiande CNS zhiliao:CNS2(baokuozhongshulinjinbuweiqinfanzhe)、CNS3 bingrenweichizhiliaoqijianzengjialiangciqiaozhu。T-LBLweichizhiliaoqijianmei 4 zhouqiaozhu 1 ci,gong 12 ci;B-LBL mei 8 zhouqiaozhu1 ci,gong 8 ci。zhu:annianlingsanlianqiaozhujiliang(jianfubiao 3)。5.zuowanjinrundezhiliaoyuanze(1)dangzhenduanshicunzaizuowanwutongxingzhongdadengdianxingzuowanjinrundezhengzhuangshi,buyidingbixuxinghuojian,ketongguo B chaoquezhen。ruojingguoyoudaojigongguzhiliaohouzuowanzhongwuxiaoshi,B chaojianchazuowanshizhihuishenghuifuzhengchangkebufangliao。(2)ruojingguoyoudaojigongguzhiliaohourengyouchixucunzaidezuowanzhongwu,xuxinghuojian。jingbinglizhengshirengcunzaizuowanjinrunzhe,yugongguzhiliaoM houxuxingshuangcezuowanfangliao,jiliangwei 18-24Gy。(er)hualiaofangan1.chushiyoudaohualiaofangan:VDLP fangan+liangliaocheng CAM fangan。(1)VDLP fanganyoudaozhiliao:changchunxinjian(VCR)1.5 mg/(m2·d),meizhou 1 ci,gong 4 ci,meicizuidajueduiliangbuchaoguo 2 mg,d8、d15、d22、d29,gong 4 ci;rouhongmeisu(DNR)25 mg/(m2·d),jingmaidizhu 6 xiaoshiru,meizhou 1 ci,d8、d15、d22、d29,gong 4 ci;zuoxuanmendongzuoanmei(L-Asp)5000 u/(m2·d),d8、d11、d14、d17、d20、d23、d26、d29,Q2d,gong 8 ci,jirouzhushe;ruo L- ASP guominkeyupeimendongmei(PEG-ASP)2500 IU/m2,jirouzhushe,d9,d23,gong 2 ji;ponisong(PDN)60 mg/(m2·d),d1~d28,koufu,di 29~35 tiandijianzhiting。PDN duiyuzhongliufuhedadehuanzheqishijiliang[(0.2~ 0.5mg/(kg·d)]kaishiyizhouneizhujianzengzhi 60mg/(m2·d),yimianfashengzhongliurongjiezonghezheng。danlian MTX qiaozhu,d1;sanlianqiaozhu(TIT),CNS1:d15、d33;CNS2、CNS3:d8、d15、d22、d33。(2)CAM fangan:ruohuanerwufare、wuyanzhongganran;ANC≥0.5×109/L,PLT≥50×109/L shi,keyu d36 kaishidiyilun CAM fangan。gong 2 geliaocheng CAM fangan,jutiyaowujianxia:huanlinzuoan(CTX)1000 mg/(m2·d),1 ci,d1,shuihuajianhua 3 tian;meisina 400mg/(m2·ci),yujingmaidizhu CTX de 0h、4h、8h,d1;atangbaozuo(Ara-C)75 mg/(m2·d) ,d3~d6,d10~d13,gong 8 tian;6-zuojizuozuo(6-MP)60 mg/(m2·d),gong 14 tian。sanlianqiaozhu,d10。xiuliao 1-2 zhou;ANC≥0.5×109/L,PLT≥50×109/L;wufarejiyanzhongganran;kaishidi 2 lun CAM。2.huanjiehougongguzhiliao(1)diweizuhezhongweizugongguzhiliao M fangan:CAM2 jieshuhou 1-2 zhou,wufare、wuyanzhongganran;jizuoqingchulvzhizhengchang;gangongneng ALT/AST≤10 beizhengchangshangxianzhi;danhongsu≤3 beizhengchangshangxianzhi,ANC≥0.5×109/L,PLT≥50×109/L shikeyikaishigongguzhiliaofangan M。CAM2 houxuexianghuishengjifuchaguchuan,bingxingliuzaopinggu,weijianmingquecanliubingzao,keyikaishi M fanganhualiao。jutiyaowuruxia:dajiliangjiaanzuozuo(MTX)3~5 g/(m2·d),meiliangzhou 1 ci,gong 4 ci[T-LBL:5 g/(m2·ci);B-LBL:3g/(m2·ci)],d8、d22、d36、d50;siqingyesuangai(CF)15 mg/m2,6 xiaoshi 1 ci,3~8 ci,genju MTX xueyaonongdugeiyudiaozheng。6-MP 25 mg/(m2· d),buchaoguo 56 tian,genju WBC diaozhengjiliang。sanlianqiaozhu:d8、d22、d36、d50。yingyongdajiliang MTX qijianxuyaojinxingshuihua、jianhua,bingjiance MTXxueyaonongdu。45 xiaoshicha MTX nongduxiaoyu 0.25μmol/L weizhengchang。ruoMTX gaoyuzhengchang,genjunongdudiaozheng CF yonglianghecishu(jianfubiao 4)。(2)gaoweizugongguzhiliaofangan:CAM2 houxuexianghuishengjifuchaguchuan,bingxingliuzaopinggu,anzhaoweixiandufenzujinrugaoweizubingrenkeyijinrugongguzhiliaogaowei(HR)fanganhualiao。CAM2 jieshuhou 1~2 zhou,wufare、wuyanzhongganran;jizuoqingchulvzhizhengchang;gangongneng ALT/AST≤10 beizhengchangshangxianzhi;danhongsu≤3beizhengchangshangxianzhi;ANC≥1×109/L,PLT≥100×109/L shikeyikaishi HR fangan,shuncibaokuo HR-1、HR-2、HR-3 fangan。① HR-1 fangan:DXM 20mg/(m2·d),koufuhuojingmaituizhu,d1~d5;VCR 1.5 mg/m2/ci(zuida 2 mg),jingmaituizhu,d1,d6;HD-MTX 5 g/(m2·ci),jingmaidizhu,d1;CF 15 mg/(m2·ci),6 xiaoshi 1 ci,3~8 ci,genju MTX xueyaonongdudiaozheng;CTX 200 mg/(m2·ci),12 xiaoshi 1 ci,jingmaidizhu,d2~d4,gong 5 ci,HD-MTX jieshuhou 7h kaishiyu;meisina 70 mg/(m2·ci),yujingmaidizhu CTX de 0h、4h、8h;Ara-c 2000 mg/(m2·ci), 12 xiaoshi 1 ci,d5,gong 2 ci;L-ASP 25000 u/(m2·d)jingmaidizhu 2 xiaoshiyishang,d6、d11;ruguo L-ASP guomin PEG-ASP 2500 u/(m2·ci),jirouzhushe,d6;TIT d1。② HR-2 fangan:DXM 20 mg/(m2·d),koufuhuojingmaituizhu,d1~d5;changchundixin(VDS)3 mg/(m2·ci),jingmaituizhu,d1,d6;HD-MTX 5 g/m2/ci,jingmaidizhu,d1;CF 15mg/(m2·ci),6 xiaoshi 1 ci,3~8 ci,genju MTX xueyaonongdudiaozheng;yihuanlinzuoan(IFO)800 mg/(m2·ci),jingmaidizhu,12 xiaoshi 1 ci,d2~d4,gong 5 ci,HD-MTX jieshuhou 7h kaishiyu;meisina 300 mg/(m2·ci),jingmaidizhu,IFO 0h、4h、8h;DNR 30 mg/(m2·ci),jingmaidizhu,d5;L-ASP 25000 u/(m2·ci),jingmaidizhu 2 xiaoshiyishang,d6、d11;ruguo L-ASP guomin PEG-ASP 2500 u /(m2·ci),jirouzhushe,d6;TIT d1。③ HR-3 fangan:DXM 20 mg/(m2· d),koufuhuojingmaituizhu,d1~d5;Ara-c 2000 mg/(m2·ci),jingmaidizhu,12 xiaoshi 1 ci,d1~d2;yituobozuo(VP-16)100 mg/(m2·ci),jingmaidizhu,12 xiaoshi 1 ci,gong 5 ci,d3~d5;L-ASP 25000 u/(m2·ci),jingmaidizhu 2 xiaoshiyishang,d6、d11;ruguo L-ASP guomin PEG-ASP 2500 u/(m2·ci),jirouzhushe,d6;TIT d5。3 liaocheng HR fanganhouxinggusuiheliuzaopinggu,CR debingrenzhihouzaizhongfu HR-1、HR-2、HR-3 fangan,jiyu MTX nongdujiancedesiqingyesuanjiejiujianfujian 3。ruguoyouliuzaojinzhanhuochuxianxinfaliuzaoxuchuzu,gaiyongerxianfanganzhiliao。3.yanchiqianghua I zhiliao(1)VDLD fangan:danggonggufanganjieshuhouyue 2 zhou,wufare、wuyanzhongganran;ANC≥1×109/L,PLT≥100×109/L shikekaishicifanganhualiao。benliaochenghualiaoqianfuchagusui,bingxingliuzaopinggu。jutiyaowuruxia:VCR 1.5 mg/(m2· d),meizhou 1 ci,diweizuhezhongweizugong 3ci,D1、8、15;gaoweizugong 4 ci,d1、d8、d15、d22,meicizuidajueduiliangbuchaoguo 2 mg;DNR huoameisu(ADR)25 mg/(m2· d),meizhou 1 ci,diweizuhezhongweizugong 3 ci,d1、d8、d15;gaoweizugong 4 ci,d1、d8、d15、d22;L-asp 10000 u/(m2·d),jirouzhushe,Q2d,d1、d4、d7、d10,gong 4 ci;L-ASP guominkeyupeimendongmei(PEG-ASP)2500 IU/(m2· ci),jirouzhushe,d4,gong 1 ji。DXM 8~10mg/(m2· d),d1~d7,d15~d21;sanlianqiaozhu:d1、d15(jin CNS3 zhezengjia 2 ci IT)。(2)CAM fangan: xiuliao 1~2 zhou;ANC≥1×109/L,PLT≥100×109/L;wufarejiyanzhongganran;jizuozhizhengchang;kaishi CAM fanganhualiao。jutiyaowuruxia:huanlinzuoan(CTX)1000 mg/(m2· d),1 ci,d1,shuihuajianhua 3 tian;meisina 400 mg/(m2·ci),yujingmaidizhu CTX de 0h、4h、8h,d1;atangbaozuo(Ara-C)75 mg/(m2· d),d3~d6,d10~d13,gong 8 tian;6-zuojizuozuo(6-MP)60 mg/(m2· d),gong 14 tian。sanlianqiaozhu,d3。xiuliaoyue 2 zhou,wufarejiyanzhongganran;ANC≥0.2×109/L,PLT≥50×109/L;kekaishiweichizhiliao。4.zhongjianweichizhiliao。zhongweizubingrenwanchengyanchiqianghuaIhoujinru 8zhouzhongjianweichizhiliao(jiyong 8 zhou 6-MP+MTX/VD fangan,jutifanganwei:6-MP 50 mg/(m2·d),chixushuiqiankongfukoufugong 8zhou;MTX 20 mg/m2,meizhou 1 ci,gong 8 ci,koufuhuojirouzhushe,genju WBC diaozhengfanganzhong6-MP dejiliang。VCR 1.5 mg/(m2· d),1 ci,d1,meicizuidajueduiliangbuchaoguo 2 mg;DXM 6mg/(m2· d),d1~d5。sanlianqiaozhu,d1。5.yanchiqianghuaⅡzhiliao(1)VDLD fangan:zhongweizubingrenwanchengzhongjianweichizhiliaohoufuchagusui,bingxingliuzaopinggu,chuyuwanquanhuanjiezhuangtai,jinruyanchiqianghuaⅡzhiliao。danghuanerwufare、wuyanzhongganran;ANC≥1×109/L,PLT≥100×109/L shikekaishicifanganhualiao。jutiyaowuruxia:VCR 1.5 mg/(m2· d),meizhou 1 ci,gong 3 ci,d1、d8、d15,meicizuidajueduiliangbuchaoguo 2 mg;DNR huoameisu(ADR)25 mg/(m2· d),meizhou 1 ci,gong 3ci,d1、d8、d15;L-asp 10000 u/(m2· d),jirouzhushe,Q2d,d1、d4、d7、d10,gong 4 ci;L-ASP guominkeyupeimendongmei(PEG-ASP)2500 IU/m2,jirouzhushe,d4,gong 1 ji。DXM 8-10mg/(m2· d),d1~d7,d15~d21;sanlianqiaozhu:d1。(2)CAM fangan: xiuliao 1~2 zhou;ANC≥1×109/L,PLT≥100×109/L;wufarejiyanzhongganran;jizuozhizhengchang;kaishi CAM fanganhualiao。jutiyaowuruxia:huanlinzuoan(CTX)1000 mg/(m2· d),1 ci,d1,shuihuajianhua 3 tian;meisina 400dmg/(m2· ci),yujingmaidizhu CTX de 0h、4h、8h,d1;atangbaozuo(Ara-C)75 mg/(m2· d) ,d3~d6,d10~d13,gong 8 tian;6-zuojizuozuo(6-MP)60 mg/(m2· d) ,gong 14 tian。sanlianqiaozhu,d3。6.weichizhiliaofangan:diweizuhegaoweizubingrenwanchengyanchiqianghuaIhoujinruweichizhiliaojieduan。ji 6-MP+MTX fanganqijianmei 4 zhoucharu VDfanganyici。kaishiweichizhiliaoshixingliuzaopinggu,weichizhiliaoqiansigexunhuanmei 8zhoupingguyici(baokuogusuijiancha),yihoumei 8 zhouxiaopingguyici(jiandanyingxiangxuejiancha,wuguchuan、yaochuan),mei 16 zhoudapinggu 1 ci(baokuonaojiyehegusuijiancha)。(1)6-MP+MTX fangan:6-MP 50 mg/(m2· d),chixushuiqiankongfukoufu;MTX 20 mg/m2,meizhou 1 ci,koufuhuojirouzhushegenju WBC diaozhengfanganzhongdeyaowujiliang。(2)VD fangan:VCR 1.5 mg/(m2·d),1 ci,meicizuidajueduiliangbuchaoguo 2 mg;DXM 6~8 mg/(m2· d),d1~d5。- 24 -zongzhiliaoliaocheng: yue 2.5 nian。(san)LBL fufahoudezhiliaoceluedayue 10%~20%jinzhanqi LBL shunanzhihuofufabingli。huanjiehouyidanfufa,wangwangbingqingjiqixiongxian,xunsuquanshenduozangqizhuanyi,zaicihuanjiekunnan,yuhoujicha,shengcunlvjinwei 10%~30%。bujiuzhiliaozhuyaobaokuozaiciyoudaohezaoxueganxibaozhichideqianghuazhiliao。bujiudemubiaoshijinkuaidadaowendingdeercihuanjie(second complete remission, CR2),jinzaoxingzaoxueganxibaoyizhi(haematopoietic stem cell transplant,HSCT)。wanjiuhualiaochangyongdeerxianzhiliaodexibaoduleiyaowuyouyihuanlinzuoan、qujiayangrouhongmeisu、kabodeng。xianyoushujubiaomingfufa LBL weijing HSCT jihuwuzhiyudejihui,erxiangbizitiHSCT(autologous HSCT,auto-HSCT),yijiyin HSCT(allogenic-HSCT,allo-HSCT)suiyoujiaogaodezhiliaoxiangguansiwang,danjuyougenggaodewubingshengcunlv,auto-HSCT fufalvmingxiangaoyu allo-HSCT。1.T-LBL wanjiuzhiliao(1)nailabin(Nelarabine,Arranon):shituoyangniaozuoleisiwu 9-b-D-atangzuozuotangniaozuozuo(ara-G)deqiantiyaowu,dui Tlinbamuxibaode DNA hechengjuyouxuanzexingyizhizuoyongerzhixibaosiwang。shiyizhongyouxiaode T xibaoteyixingxibaoduyaowu,yongyuzhiliaonanzhi、fufade T-LBL/ALL,takedanyaozhiliaohuoyuqitaxibaoduyaowulianyong。ertongyingyongjiliang 650 mg/(m2· d) ×5 d,21d yiliaocheng,Ⅰ/Ⅱqiertongjichengrenyanjiu,danyaofanyinglv 14%~55%。changjiandefuzuoyongweijiliangyilaixingshenjingduxing,biaoxian 2 jihuo 3 jiganjue、yundongshenjingbingbianhejirouguzuotengtong,youdebingrenbiaoxiantoutong、shishui、ganjuechidun、zuozuofazuo、gongjishidiaodeng,duoweikenixingde。qitafuzuoyongweixueyeduxing、exinoutudengxiaohuadaofanying。nailabinzhiliaofufa/nanzhi T-ALL/LBL zuichangyongdelianhehualiaoshihuanlinzuoanjiayituobozuo,Leah dengbaodao 5/7 CR,qizhong 4 lideyijinxing HSCT,danjin 1 licunhuo,qitabingrensiyujibingjinzhanhezhiliaoxiangguanhebingzheng。chuliaolianhehualiao,K Burley baodao 1 liyizhihoufufadeT-ALL caiyongnailabinjiagongzhexibaohuishudadaochixu CR。Annalisa dengtichukeyigenjubingrenjiyinbiaodapu,banailabinxuanzexingdilianhe PI3K、Bcl2、MEK yizhiji,yizengjiaqiliaoxiao。(2)daleimudankang(Daratumumab):CD38 renyuanhuadankang,CD38kangyuanzaihualiaoqianhouhuozaifufahouT-ALL/LBLxibaoshangbiaodawending,kenengchengweilianghaodezhiliaobadian。bingkezuowei CAR-T xibaozhiliaodebadian,muqianjunzaitansuozhong。(3)qita:ru PI3K yizhiji、mTOR yizhijihuo PI3K-mTORshuangzhongyizhijilianhetangpizhijisuyongyugaowei PTEN queshidebingren;lusuolitini(ruxolitinib):JAK/STAT tongluyizhiji,nenggoukefutangpizhijisunaiyaodengjunzaiyanjiuzhong。2.B-LBL wanjiuzhiliao(1)laoansuanjimeiyizhiji(TKI):keyongyuzhiliaochuzhihefufade Ph+ALL / LBL,tongguobaxiangzuoyongyu BCR/ABL ronghejiyincongerfahuiliaoxiao。yimatinidanyaoyouxiaolvda 70%,lianhehualiao CR lvkeda90%。dierdaidashatiniheniluotinikeyongyuduiyimatininaiyaodebingren,juyoutouguoxuenaopingzhangdetexing,liaoxiaoyouyuyimatini。(2)CD19 teyixingqianhekangyuanshouti T xibao(CAR T xibao):zhiliaofufa/nanzhi B-ALL/LBL CR lvdadao 70%~92%,liaoxiaoxianzhu,huanjiehoukeqiaojieyizhi,CAR-T xibaozhiliaodeduxingweixibaoyinzishifangzonghezhengheshenjingduxing。(3)qitarushuangteyixingkangti(Blinatumomab)、pengtizuomi(Bortezomib)deng,zhiliaofufa/nanzhiALL/LBLjunyouyidingliaoxiao。(si)zaoxueganxibaoyizhi1.duihualiaoguochengzhongduogepinggudianxianshiduihualiaobumingan,zhongqipingguyou mingquecan liubing zao;huo cunzaiyu houbu liangjiyin :juyout(9;22)/BCR-ABL1、MLL zhongpaideng,keangaoweizufanganhualiao,wanchengsanliaochenggaoweifanganbaokuo HR-1、HR-2、HR-3 houxingyijiyinzaoxueganxibaoyizhi。2.duiyuyuanfaliuzaofufahuogusuifufahuozhiliaozhongjinzhandebingrentongguowanjiuzhiliaonenggoudadaowanquanhuanjiehuobufenhuanjiehuodeyizhijihuizhe,xujinkuaixingyijiyinzaoxueganxibaoyizhi。liu、hebingzhengdechuli(yi)duicunzaishangqiangjingmaiyapozonghezheng、qidaogengzudebingrendechuliyuanzedayue 10%T-LBL kenengchuxianyanzhongdeqidaogengzu(banhuobubanshangqiangjingmaiyapozonghezheng),weizhenzhengzhongliujizheng。1.duicileibingrenshangweijingbingliquezhenzhe,jinjiyingyongquanshenmazuishoushuhuojian。2.kexianyuxiaohualiao(ruponisong 60 mg/m2koufuhuo VP fangan)huanjiehuxikunnan,yuyongyaohou 24~48 xiaoshineizhengzhuangkongzhihoujinzaoxingbinglijiancha,bingyingxuanzeqinxixingzuixiaodecaozuoquezhen,keyikaolvqucaishunxuwei:gusui、xiongfushui、qianbiaolinbajie、pifuhuoqitaqianbiaokeyiliuzao、shenbulinbajiehuozhongwuchuancihuojian、liuzaoshoushuquhuojian。hualiaoqianxuqianshuweiquezhenhualiaoxieyishu,gaozhijiachanghualiaokenengyingxiangbinglijieguoerwufaquezhen。3.duibanyoushangqiangjingmaiyapozonghezhengdebingrenyingbimianshangzhishuye。(er)zhongliurongjiezonghezhengdechulicunzaigaozhongliufuhede、hualiaoqianyiyougaoniaosuanxuezheng、shengongnengsunhai、shaoniaoshuizhongdebingrenweifashengzhongliurongjiezonghezhengdegaoweirenqun,xucaiqujijideyufanghezhiliaocuoshi。1.yanmijiancegexiangshengmingtizheng、churuliang、tizhong、xuedianjiezhi、ganshengongneng。2.yingyongbiezuochunjianshaoniaosuanshengcheng:10mg/(kg· d)[300mg/(m2· d)],fen2~3cikoufu。biyaoshiyingyongniaosuanyanghuamei0.1~0.15 mg/(kg·ci)kexunsujiangdiniaosuanshuiping。3.jijishuihualiniao:3000 ml/(m2· d)[125 ml/(m2· h)],yong1/4~1/3zhangyeti,yibanbuyonghanjiaye。geiyusuniao0.5~1mg/(kg·ci),Q12hliniao,baozhengchurupingheng,weichiniaoliangdayuezai125 ml/(m2· h)。buchongzugouyeti,kebubijianhua,yifangjianhuatiaojianxialinsuanyanhehuangzuozuozaishenxiaoguanchenjijinyibujiazhongshensunshang。(san)duiyoushenjingxitongqinfanhuoyapodebingrenchuliyuanzejinkuaiwanshanjiancha,jinzaohualiao,yifangnao、jisuihuozhouweishenjingyapohuoqinfanguojiu,zaochengshenjingxitonggongnenghuifuyanchishenzhibukenidesunshang。bingan CNS2、CNS3 geiyuqiaozhu。qi、zhiliaozhongqitazhuyishixiang(yi)hualiaohoujiancexuechanggui,ANC <0.5×109/L,PLT<100×109/L,kepixiazhushelixibaocijiyinzi(G-CSF)5μg/(kg·d)zhiANC>1×109/Ltingyong。(er)hualiaoyaowudufufanyingdepandingbiaozhunan NCI buliangfanyingdefenjibiaozhun(CTCAE version 4.0,2009)jiance,hualiaoqianhoujunxuzhuyijiancexindiantu、chaoshengxindongtu、ganshengong、niaochanggui、xuedianfenmei、ningxuegongnengdengjiayiyufang。(san)yufangkashifeinangchongfeiyanchangguikoufufufanghuanganyizuo[25 mg/(kg· d),meitian 2 ci],meizhoufu 3 tianting 4tian,hualiaokaishizhidaohualiaojieshuhou 3geyue。HD-MTXyongqian 24 xiaoshidaoyong MTX houzhishao 72 xiaoshitingyongfufanghuanganyizuo。ba、tingyaohousuizhenshijianjipingguneirong(yi)shoucipinggu(tingyao 3 geyue)fuchagusuichanggui、jiyinhe MRD;liuzaopinggubaokuo B chao、CT deng,zuihaozuo PET/CT;fuchamianyigongnengjixindiantu、xinzangcaichao;fuchaganshengong、LDH。(er)tingyaodi 1、2 nianmei 3 geyue 1 cijiandanpinggu,baokuoguchuanheliuzaodeyingxiangxuejiancha(B chaohe CT pingsao)jigangonghe LDH。mei 6 geyue 1 cidapinggu,baokuoguchuanheliuzaopinggu:xiangguan B chao、zengqiang CT jianchahuo MR、mianyigongneng、gangonghe LDH deng。(san)tingyaodi 3 nianyihoumeibannianpinggu 1 ci,zhuyaozuoguchuanheliuzaodeyingxiangxue(B chaoheCT pingsao)jigangonghe LDH。shiqingkuangjiazuoneifenmijisujizhishangdengjiancha。jiu、zhuanzhentiaojian(yi)dangdiyiyuanmeiyouzhenliaotiaojian、meiyouzhiliaojingyandexujiangbingrenzhuanzhenzhiyouzhiliaotiaojiandeshangjiyiyuanyibaozhengliaoxiao。(er)juyougaozhongliufuhehuozhongliuhebingzhengdeweizhongbingrenxuzhuanzhenzhiyoujingyandeshangjiyiyuanyiwanjiushengming。(san)jingshangjiyiyuanzhiliaohouwending、dadaowanquanhuanjiedebingrenkezhuanzhenzhiyouzhiliaotiaojiandexiayijiyiyuanjixuzhiliao,liruweichizhiliaodebingren。qijianjianyidingqihuishangjiyiyuanjinxingliuzaopinggu。ruweichiqijianfashengyanzhongdehebingzheng,ruyanzhongganran,kezhuanhuishangjiyiyuanjinxingqiangjiu。fu ertonglinbamuxibaolinbaliuzhenliaoguifan(2019 nianban)bianxieshendingzhuanjiazu(anxingshibihuapaixu)zuchang:wangtianyouchengyuan:wangtianyou、sunxiaofei、zhangyonghong、tangjingyan、jinling、yangzuo、duanyanlong、zhouchunju、gaozifen、dixiaowen
接(闯颈别)着(窜丑耻辞)就(闯颈耻)会(贬耻颈)打(顿补)开(碍补颈)聊(尝颈补辞)天(罢颈补苍)记(闯颈)录(尝耻)空(碍辞苍驳)间(闯颈补苍)管(骋耻补苍)理(尝颈),在(窜补颈)这(窜丑别)里(尝颈)就(闯颈耻)可(碍别)以(驰颈)看(碍补苍)到(顿补辞)我(奥辞)们(惭别苍)微(奥别颈)信(齿颈苍)好(贬补辞)友(驰辞耻)里(尝颈)面(惭颈补苍)的(顿别)所(厂耻辞)有(驰辞耻)聊(尝颈补辞)天(罢颈补苍)记(闯颈)录(尝耻)了(尝颈补辞),我(奥辞)们(惭别苍)随(厂耻颈)便(叠颈补苍)点(顿颈补苍)击(闯颈)一(驰颈)个(骋别)好(贬补辞)友(驰辞耻)打(顿补)开(碍补颈),都(顿耻)可(碍别)以(驰颈)看(碍补苍)到(顿补辞)我(奥辞)们(惭别苍)给(骋别颈)好(贬补辞)友(驰辞耻)聊(尝颈补辞)天(罢颈补苍)时(厂丑颈)发(贵补)送(厂辞苍驳)的(顿别)每(惭别颈)一(驰颈)张(窜丑补苍驳)图(罢耻)标(叠颈补辞),以(驰颈)及(闯颈)接(闯颈别)收(厂丑辞耻)的(顿别)每(惭别颈)一(驰颈)张(窜丑补苍驳)图(罢耻),每(惭别颈)一(驰颈)段(顿耻补苍)视(厂丑颈)频(笔颈苍),是(厂丑颈)不(叠耻)是(厂丑颈)都(顿耻)充(颁丑辞苍驳)满(惭补苍)了(尝颈补辞)回(贬耻颈)忆(驰颈),除(颁丑耻)此(颁颈)之(窜丑颈)外(奥补颈),我(奥辞)们(惭别苍)点(顿颈补苍)击(闯颈)左(窜耻辞)上(厂丑补苍驳)角(闯颈补辞)的(顿别)【全(蚕耻补苍)部(叠耻)时(厂丑颈)间(闯颈补苍)】
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