体育外围

消化内科二区

department of ent

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多发性骨髓瘤的诊治思路

时间:2013-05-31       来源:体育外围

体育外围 duofaxinggusuiliuweifashengyublinbaxibaodeexingjiangxibaobing。haofayuzhonglaonian,danjinnianfabinglvyouzenggaojifabingnianlingyoutiqianqushi。

病因与发病机制不清楚。可能与电离辐射、慢性抗原刺激、EB病毒或卡氏肉瘤相关的疱疹病毒感染诱发C-MYC、N-RAS或K-RAS或H-RAS等癌基因高表达有关。也可能与一些细胞因子有关。IL-6为多发性骨髓瘤的生长和分化因子。溶骨性病变的发生可能与IL-1、IL-6、TNF等溶骨因子有关[1]

本病误诊率很高,患者可因发热、尿改变、腰腿痛被误诊为呼吸系统感染、肾炎、骨病而延误病情[1]体育外围。在诊治中应予足够重视。

一.易误诊的一些临床表现[2]:

(yi)ganran

youyuzhengchangmianyiqiudanbaijianshao,yichangmianyiqiudanbaizengduodanwumianyihuoxing;baixibaojianshao、pinxuejifanghualiaodengyingxiangzhengchangmianyigongneng,guyiyufanfuganran。huanzhekeyifarezuoweishoufazhengzhuangjiuzhen。yifashengshanggan、denghuxidaoganranhuominiaoxitongganran,nvxinghuanzhegengyiyufashengminiaoxitongganran。zaibingchengwanqi,ganranshizhisidezhuyaoyuanyinzhiyi。

guduiyuzhonglaonianhuanzhefanfufashengganran,buyingjinjuxianyukangganranzhiliao,yingzaikangganranzhiliaodetongshi,jijixunzhaoshifouyouyuanfabing。ruohuanzhehebingyougutong、pinxue、chuxuedengyingkaolvdaobenbingdekeneng。

(er)gusuiliugubing

体育外围 gutongherongguxingguzhipohuaishibenbingdetuchulinchuangbiaoxian。gutongbuweiyiyaobeibuzuiduojian,qiciweixionggu、leiguhexiazhiguge。

体育外围 youyubenbinghuanzhechangyouyanzhong,changzaishaoyonglihuobujingyiqingkuangxiajikefashengbinglixingguzhe,kezaochengdejixiexingyapoyinqishenjingxitongzhengzhuang,yanzhongzhezaochengjietan。ruojietanshijianguojiujishijingguoyouxiaozhiliao,huanzheyehennanhuifuxingzou,jidadiyingxianghuanzhedeshengcunzhiliang。

yekechuxiangugezhongwu,liuxibaoyougusuixiangwaijinrunguzhi、gumojilinjinzuzhixingchengzhongkuai。

juxianyuyigebuweidegulixingjiangxibaoliujiaoshaojian,zuizhongkeyanbianweiduofaxinggusuiliu。

体育外围 xxianjianchazaibenbingzhenduanzhongjuyouzhongyaoyiyi。yangxingbingbianbuweizhuyaozailugu、gupen、leigu、jizhuigu,yekejianyusizhigu。dianxingbiaoxianweiyixiasanzhong: ①chuanzaoyangrongguxingbingbian,weiduofaxingleiyuanxingtouliangqu,weibenbingdeteshuxxianbiaoxian。yilugu、gupenzuiyifaxian。②mimanxingguzhishusong。③binglixingguzhe,zuichangjianyuxiaxiongzhuijishangyaozhui,yejianyuleigudengchu。duiyisibinglikexinghecigongzhenjianchayimingquerongguxingbingbiandebuwei。

huanzhekeyingutonghuoyaotuitongbuyuzhongshi,huojiuzhenyuguke,beiwuzhenniushang、guzhe、gujiehehuoguzhongliueryanwubingqing。

体育外围 guduiyuzhonglaonianhuanzheyanzhongguzhishusonghuofashengguzhedehuanzhe,zaizhenduanshiyingkaolvdaobenbing。zhuyichaxuemianyiqiudanbaihexueqingdanbaidianyongdengyizaoqimingquezhenduan。

(san)gusuiliushenbing

体育外围 shenzangbingbianweibenbingchangjianerzhongyaodebingbian。linchuangbiaoxianyidanbainiaozuichangjian,qiciweixueniao。wanqikefazhanweimanxingshengongnengbuquanhuoniaoduzheng,weibenbingzhisidezhuyaoyuanyinzhiyi。

guowaiyanjiufaxian,mmshenbinghuanzhezaibinglishangshenxiaoqiubingbianjiaoqing,erxiaoguan-jianzhibingbianyanzhong,guhuanzhelinchuangshanghenshaochuxiangaoxueya。

gusuiliushenbingyouduozhongyinsusuozhi,qingliandanbainiaoduishenxiaoguandesunshangheqinglianzaishenxiaoqiudechenjiyinqidedianfenyangbianshizhuyaoyuanyin,ciwaigusuiliuxibaojinrun、gaoxuegai、gaoniaosuanxuezhengdengyecanyufabing。

mianyifenxingyushensunhaiguanximiqie,qinglianxingshensunhailvzuigao。

体育外围 huanzhekeyinxueniaohuodanbainiaojiuzhenyushenkehuozhongyike,beiwuzhenweishenyandengerzhangqidebudaoyouxiaozhiliao,shibingqingjinzhanzhiwanqihuofazhanzhiniaoduzheng。

guduiyuzhonglaonianhuanzhezhangqidanbainiao、xueniaobunengmingquezhenduanzhe,yingjishizuoshenhuojian、gusuichuancihuogusui、xuemianyiqiudanbaihexueqingdanbaidianyongdenggusuiliuxiangguanjiancha。

er.zhenduanyujianbiezhenduan:

由于本病患者早期可无骨痛、贫血或血常规、尿常规改变,诊断主要依据以下三方面实验室检查[1]:

(yi)zhenduanbiaozhun:

1.gusuijiangxibao>10%,bingyouyichangjiangxibao(gusuiliuxibaozhuyaoweiyuanshijiangxibaohuoyouzhijiangxibao)huozuzhihuojianzhengshiweigusuiliuxibao;

2.血清中出现大量单克隆免疫球蛋白(单克隆免疫球蛋白可在血清蛋白电泳的γ区或β区或α2区出现一窄底高峰,又称M蛋白): IgG>35g/L,IgA>20g/L,IgD>2.0g/L,IgE>2.0g/L,IgM>15g/L,或尿单克隆免疫球蛋白轻链(即尿本周氏蛋白)>1.0g/24h;

3.wuqitayuanyinderongguxingbingbianhuoguangfanguzhishusong。

体育外围duiyuyoutiaojiandeyiyuanyingjinyibuzuoxialiejiancha:

(1)mianyifenxing: yonghuanzhegusuijinxingjiancha,cd10、cd19、 cd20dengbxibaobiaojidankang、 cd38 、cd138jiangxibaobiaojidankangbiaodazenggao。

(2)xibaoyichuanxuejiancha: duobiaoxianwei14q+、del(14)、t(11;14);

(3)β2体育外围微球蛋白: 血或尿中均可升高,为预后不良的因素。特别是血的含量,不受肾功能的影响。

(er)fenxing:

zailinchuangshang,genjuxueqingzhongmchengfendetediankebabenbingfenweiigg、iga、igd、ige、igm、qinglianxing、shuangkelongxinghebufenmixingdeng8zhongleixing,qizhongyiiggxingzuichangjian,qiciweiigaxing。

(san)jianbiezhenduan:

1.fanyingxingjiangxibaozengduozheng: jianyujiehe、shanghan、zishenmianyibingdeng,yibangusuijiangxibaobuchaoguo10%,qiejunweichengshujiangxibao。

体育外围2.qitachanshengmdanbaidejibing: manxingganbing、zishenmianyibing、exingzhongliurulinbaliudengkechanshengshaoliangmdanbai。

体育外围3.yiyiweimingdedankelongmianyiqiudanbaixuezheng(mgus): xueqingzhongmdanbaidiyu30g/l,gusuizhongjiangxibaodiyu10%,wurongguxingbingbian、pinxue、gaogaixuezhengheshengongnengbuquan。mdanbaikeduonianwubianhua。yue5%dehuanzhezuizhongfazhanweiduofaxinggusuiliu。

4.guzhuanyiai: duobanchengguxingcheng,rongguxingquesunzhouweiyougumiduzengjia,qiexueqingjianxinglinsuanmeimingxianshenggao。youyuanfabingzaocunzai。

san.zhiliao

体育外围 (yi)zhiliaoyuanze: wuzhengzhuangwendingqigusuiliuwuxuzhiliao,dingqisuifang;xuehuoniaozhongmdanbaijinxingxingshenggaohuochuxianlinchuangzhengzhuangzhe,bixuzhiliao。nianlingxiaoyu70suidehuanzhe,ruotiaojianyunxujinliangjinxingzaoxueganxibaoyizhi。

duiyudaduoshuzhiliaoyouxiaodegusuiliuhuanzhe,mdanbaidengzhuyaozhibiaozaiyidingshijianneiquyuwending,jinrupingtaiqi,kejiyumianyizhiliao、dongtaiguanchadeng。

(er)yibanzhiliao:

1.jiankangxuanjiao: ① gusuichuancishizhenduanbenbingbibukeshaodejianchazhiyi,guchuanduirentiwushanghai,gaolinglaonianhuanzheyekejinxing;②benbingfazhanburujixingbaixuebingxiongmeng,dabufenhuanzhejingyouxiaozhiliaobingqingkededaokongzhi;③yongyuzhiliaobenbingdehualiaofanganbijiaowenhe,exin、outudengweichangdaofanyingqingwei,dabufenhuanzhejunnengnaishou;④gaibingruobujishizhenzhi,fazhanzhiyanzhonggubingshenzhijietanhuoniaoduzheng,jianghuijihuanzhejijiashudailaijidadetongkujijingjifudan。gujiashujihuanzheyingpeiheyihurenyuanjijizhiliao。

体育外围2. yibanzhiliao: ①wujizhurongguxingbingbianzhekeshidanghuodong,youjizhubingbianzheyingxianzhihuodongliang,yimianfashengyasuoxingguzhe。②shidangbuchongdanbaizhi(youshengongnengbuquanzheyingdidanbai、gaoreliangyinshi)、weishengsu、dianjiezhideng。③hualiaoqijianjijianxieqizhuyishukouheqingxihuiyin,fangzhikouqiangganran、huiyinganranjigangzhounongzhongdechuxian。

(san)huaxuezhiliao:

体育外围1.zhuyuanhualiaofangan: mafalanhehuanlinxiananduigusuiliuxibaohualiaoliaoxiaojiaohao,dandanyaoliaoxiaoburulianhehualiao。mafalanchangyoushenzangpaixie,qieyisunshangzaoxueganxibao。guruoyoushenzangbingbianhuozhunbeixingzaoxueganxibaoyizhizhe,yingjinliangbimianhanciyaodefangan。changyongyixiahualiaofangan:

(1)VAD方案: V(长春新硷): 0.4mg/d,静注,第1~4天;A(阿霉素)9mg/m2体育外围.d,静注,第1~4天;D(地塞米松)40mg/d,静注或口服,第1~4天,第9~12天,第17~20天。每4周一疗程。多用于治疗复发、难治性骨髓瘤的治疗,有效率达30%左右。由于此方案有肾脏病变也可应用,不损伤造血干细胞,对初发骨髓瘤患者有效率达60%,故得到广泛应用。

(2)M2方案: 长春新硷1.2mg/m2,静注,第1天;卡氮芥20mg/m2,静注,第1天;环磷酰胺400mg/m2,静注,第1天;马法兰8mg/m2.d,口服,第1~4天;强的松20mg/m2.d,第1~14天。间歇5周重复1疗程。初发骨髓瘤患者有效率达60-80%。

(3)MP方案: 马法兰(M)5mg/m2.d,口服,第1~7天;强的松(P)40mg/m2.d,第1~7天。既往此方案被作为骨髓瘤治疗的金标准方案,但由于强度较弱,仅用于年老体弱的患者。有效率达55-60%。

2.chuyuanhoudezhidao:

①zuihaojudanjian,zhuyifangjianxiaodu;②wochuang,shaohuodong;③daikouzhao,shaoqugonggongchangsuo;④shidangbugai;⑤dingqifuchaxuechangguihegusuixiang,lejiebingqingbianhua,bingpanduanliaoxiao。

3.menzhenzhiliaofangan:

体育外围(1)mpfangan: yongfatongshang。youyucifanganzuhejiandan,duxingdi,yiyumenzhenshishi。

(2)口服反应停[3]: 反应停具有调节免疫和抗血管新生作用,用于本病有效率30%左右。开始剂量为200 mg/d,渐加量,一般400-600 mg/d维持,最大用至800 mg/d。不能耐受大剂量者,可100-200 mg/d,也可取得较好疗效。主要副作用为头晕、便秘、手脚麻木等临床表现。患者可门诊用药。

(3)三氧化二砷[4]: 具有促进凋亡和抗血管新生作用, 10mg静脉点滴,每日一次,28天一疗程[1]

(si)fangshezhiliao: zhuyaoyongyugulixinghesuiwaijiangxibaoliudejubufangliao。

(wu)mianyizhiliao:

1.ganraosu: α-ganraosunengtigaohuanzhedehualiaowanquanhuanjielv,yanzhangwubingshengcunlv。jiliangwei: 300wanu,pixiazhushe,getianyici。zuihaoyongbannianyishang。zhusheguochengzhong,huanzhekenengchuxianfaredengyangzhengzhuang,koufujiereyaojikehuanjie。yiyumenzhenyingyong。

体育外围2.baixibaojiesu-2huokangbaixibaojiesu-6kangti: zhuyaoyongyuqingchucanliubingzao。

(六)造血干细胞移植: 50岁以下患者应积极进行造血干细胞移植;50-70岁应酌情处理,原则上应尽量进行造血干细胞移植;70岁以上不宜进行[15]

1.zitizaoxueganxibaoyizhi: weiduofaxinggusuiliuzhiliaodeyidajinbu,qiliaoxiaomingxianyouyuchangguihualiao。qieerciyizhideliaoxiaogenghao。kexingzitiwaizhouxueganxibaoyizhi,jiagebianyi,caozuojianbian,zaoxuehuifukuai,yibeiguangfancaiyong。

2.yijiyinzaoxueganxibaoyizhi: shigenzhibenbingdeweiyifangfa,danxiangguansiwanglvgao,zhuyaoyongyuyouheshigongzhedenianqinghuanzhe。

(qi)duizhengchuli:

1.抗感染: 需选择抗G+菌和抗G-菌的抗生素,同时注意真菌感染的可能。

体育外围2.jiuzhenggutonghegaogai: jiyushuanglinsuanyan90 mg/cijingmaidiandizhishao6xiaoshi,yiyueyici。shengongnengbuquanzheshidangjianliang。

3.shengongnengbuquandezhiliao: yingdidanbai、gaoreliangyinshi;bimianganmaodengganran;bimianduishenyousunhaiyaowudeyingyong;jiuzhengbianmi、gaogaideng,jinzaoxingtouxizhiliao。

si.bingchengheyuhou:

体育外围 ziranbingcheng6-12yue,jingyouxiaohualiaohou,zhongweishengcunqikeda3nian。siwangyuanyinweiganran、chuxueheshengongnengbuquan。

预后与许多因素有关。病程晚期、肾功能不全、幼稚浆细胞多、β2微球蛋白和IL-6高水平,CD56或CD138高表达、-13或13q-等均是预后不良的因素。

cankaowenxian

1duofaxinggusuiliujiqixiangguanjibing。houjian,fuweijunzhubian,shanghaikejichubanshe,2002。

2..chenshilun,liyanchen,zhangpengdeng。798limdanbaiyangxinghuanzhedelinchuangfenxi。zhonghuaneikezazhi,2001,40: 398-400

体育外围3.singhal s,mehta j,desikan k,et al.antitumor activity of thalidomide in refractor multiple myeloma.n engl j med,1999,30;341:1565-1571

体育外围4..miller inh jr. moleculer targets of arsenic trioxide in malignentmcells.oncologist 2002;7 suppl 1:14-9

体育外围5.anderson kc,shanghnessy j d,banlogie b,et al.multipl myeloma(am soc hematol educ program),2002;21440

ciwei04nianfabiaodefangan,zhuyuanhualiaofanganyiyouxieguoshi,danzhenduansilukegongcankao。

体育外围xiexieyuedu,xiwangduininhejiarenyousuobangzhu!


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