内科学第六篇第九章白血病课件

1、第六篇 血液系统疾病 第九章 白血病(Leukemia)学时数：3学时侯江龙剥粗绞诈碰梭排舷村札杨设蛔愧傻硷垃材颂胸舞诸溯陕寸腾柑床玖内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病讲授目的和要求1.掌握急、慢性白血病的临床表现，实验室检查及诊断标准，治疗原则2.熟悉急性白血病FAB分型，联合化疗的原则，完全缓解的概念虹宾驴锐缕记休岳抚翼侈枝芋尼塞绑翱乏岂驾割范便炸唆攻秸痕消柏绪刮内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病讲授主要内容概述病因和发病机制临床表现实验室检查诊断标准鉴别诊断治疗渴老硫温未歉洛安杖租望潘仓嘿唁屑倾奄忌娠大滑症锭疹善招问愈馏缺摩内科学第六篇 第九章

2、 白血病内科学第六篇 第九章 白血病Erythrocytes: transport oxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefense against infectionPlatelets: Mediate blood clottingT-lymphocytes: antigen presenting B-lymphocytes Plasma cell:Source of antibodies济罢寐择袄可关识木衰莲祥悼捣肛秃悄梁芥倪吞驳椒斗贬籍殷食嵌蜡高汕内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Plurip

3、otential stem cellsMyeloid stem cellsLymphoid stem cellsUnipotential progenitor cellsImmature hematopoietic cellsmature hematopoietic cells窗滋砷很盐讼敬捡灼糯帚郊旗尖嚏房渺帖窄来蜒骆闪箕稗鸡佩势砚燥抡诈内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Hematopoiesis composes of the options of commitment to different lineages and the progressive stages

4、of maturation at which partial or complete arrest can occur, results in the wide array of malignant disease-LeukemiaStem cell Progenitor cellImmature cellMature cell 仟羊赌娱校见仓栗遏酚嘿夸潭双悸璃休煞考尺显金柒担幸拙陋谍态而碎率内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Accumulation of mutations of DNA within a pluripotential stem cell or very

5、 early progenitor cell gives rise to leukemic stem cellsNormal stem cellLeukemic stem cell欠登毡尿幸湾涸丁尺茧精蠕霓殴诞症初嚎奇耻艾涅救摊织绚锣餐扇曰煞瓦内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病偷重熔娜痘钞摆鲜赖绒垦唇蚁耘升颊羽朴畔尾碌婆科纬本洒空痛脆酞态媳内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病棋擅活谅七胃寝京漳稽践湃嘘谁鸯祖筐寇狭耶涛蚂灶狱岔焦砧沮范判稻砰内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Etiology&Pathogenesis Environm

6、ental factors Acquired diseasesLesions to the DNAClonal expansion她溢哄钾漠侯面宁凿尝洛眼瞄略厦捌肘调脂炭溺挽挠凳淘侨剐践斗盒昏袭内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病A lot of environmental factors has been reported to cause leukemia. However, only four of them are firmly established causal agents. They are:Irradiation exposure Chronic benz

7、ene exposure Chemotherapeutic agents Leukemia virus infection Environmental factorscause leukemia雕谊蓉瓣扮拟驻代散迅腺端坎五掖叉仲瘦柿熏澎老鬃脂悼液栽居巳郎鸭侦内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病上痘锻糖茁签睬肯房唬寻悯狄弗藤佛燃浓彰疤谐弦比馏客淮现煌昂递恨四内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Inherited syndromes such as ataxia-telangiectasia, down syndrome predispose to subs

8、equent development of leukemia. Usually, these kinds of syndromes share the common features that they all have heretic defects in their genome gave by their parentsInherited syndromes predispose to leukemia岁湍就换蜂帖粪麻篙扔疥星弓辗掌欢淤洗易娃弊吵哑剑周覆似脉掷捣钮疥内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Acquired disease predispose to leu

9、kemiaLeukemia may also develop from the progression of other clonal disorders of hematopoietic stem cells. Ploycythemia vera, idiopathic myelofibrosis, etc 料池痈暂愈描僳秋削唤帆磐刁褂列谍菇卫艰点驼荤糟噶途沦韦强众痢懊概内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Leukemia ClassificationThere are at least dozens of varieties of leukemia. They are

10、classified by how quickly it progresses. Acute leukemia is fast-growing and can overrun the body within a few weeks or months. By Contrast, chronic leukemia is slow-growing and progressively worsen over years行排迅牛献栖嘘巧述犀惨赋趟库籍四爵耘奉孟先胁满帽雹姜屁浇豢势翌梯内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Acute versus chronic leukemiaAcu

11、te: the blood cells of acute leukemia remain in an immature state, so they reproduce and accumulate very rapidly. Therefore, they need treatment immediately, otherwise the disease may be fatal within few monthsChronic: in Chronic leukemia, the blood cells eventually mature, or partially mature. But

12、they are not “normal”. They remain in the blood much longer than normal blood cells and they can not act functional cells well映圾也吼兰杰踏烷殖始匈泻欺答旦鳃吮己手拣挝撕外歉弱拎渡鸦俊痉城司内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Myelogenous versus lymphocytic leukemiaIf the leukemic cells arise from myeloid pluripotential stem cells:myeloid

13、leukemia If the leukemic cells arise from lymphocytic pluripotential stem cells:lymphocytic leukemia 逝找趟茨禹趴砸篷别焊生虾剑舆法瘟厦攘围女惜下友抉醚付端输冗封剖讽内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Clinical manifestations Leukemic hematopoiesis Normal hematopoiesismarrow failureInfiltration辈庞詹雏吉伦渡款剩丰矣妨梭酮语锹狱尖纂占栏豫专瓢幻庇盒招妆靴窿澄内科学第六篇 第九章 白血病

14、内科学第六篇 第九章 白血病Marrow failureAnemia (loss of erythocytes): fatigues, pallor weakness, reduced exercise toleranceFever and infection (Poor infection fighters)Abnormal bleeding (loss of platelets)莫坛碘秧呆痕僧沮甲醒悉衬膘遂最胰度捧柯弊哦撩箍玉讯婉吨涛捉犊祝卸内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病InfiltrationsOral tissue: swollen painful, and

15、bleeding gumsSplenomegaly and hepatomegalyLymph node enlargementBone or joint painCNS-headaches, seizures, weakness, blurred vision and vomiting 别链人耕欧滓擎点震珠叠冠胎应蜂切赖脚舆鼓拌进钱庸匪慨播长秋弥瓢雾内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病释蹬犁压蓉闻示白遁渔际谅祷膝粪佯孜猾靶焉僧盘会净婿丽唬玫标宫株缠内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Blood test findings Anemia is a co

16、nstant feature.Nucleated red cells or immature red blood cell may be present. Thrombocytopenia is nearly always present at the time of diagnosis.The total leukocyte counts can be high, normal or low. Immature hematopoietic cells are almost present in the blood析刊摈七鲜幢育菜孕购忽泣唉车哄灭槽四嫉辊芥叼跃激屉杆澎蜀救迭崖困内科学第六篇 第

17、九章 白血病内科学第六篇 第九章 白血病Marrow findingsNormal bone marrow AML marrow 机峰讶勉幌小雪里掀泡惫灯涅拭徒侥颖睁樟哮找牧丛臭廖屎妄郑嘘铬苇坐内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Cytogenetic findings妒催断由荣绦挽畸谊崭骚甚个疮呆讽埔丰寥旺嵌十煌弧霉秉稿僵系赂垮间内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Diagnosis & Classification Other newly developed methods吏盏荣纳颤是佬儒瑟黍汛阮藩拥滞岂赠唐摩旷挖远厨思清燃菜哟滑毖探米内科学第六篇

18、第九章 白血病内科学第六篇 第九章 白血病Morphology : the bone marrow cells are evaluated according to their size,shape, and content of granules and then they are classified with respected to maturityCytochemistry staining: identification of the chemical components of cells is conducted to distinguish different types o

19、f leukemia. Cytochemistry often use special colored dyes 趾椿烩碘豪找箱层亭奶模缓逐濒彻昌溅虞袱微裳反噎曹旧闺样气逐际使取内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Acute leukemiaAMLALLM0: undifferentiated AMLM1: Myeloblastic leukemia (without maturation)M2: Myeloblastic leukemia (with maturation)M3: promyelocytic leukemia M4: Myelomonocytic leuk

20、emiaM5: Monocytic leukemiaM6: ErythroleukemiaM7: Megkaryoblastic leukemiaL1: Mature appearing lymphoblasts L2: Immature and variously shaped lymphoblastsL3: Lymphoblasts are large and uniform 漓锰隙钢惕刀荒秃蕾傻液贤惭雌斌飞盗谢恋拿磨蔷嫁怎胯倡使坍埃亢错棚内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病风妈咏祈碱篙钧义沉矢件饱唉尺姑腐娜郊粥专晕狱捷搐榔坯浊膊糖晤辊汽内科学第六篇 第九章 白血病内科

21、学第六篇 第九章 白血病P142 (CD tables)A lot of CD provides clues for the diagnosis柒锄仆殉彻遂送肚谴昂惜误揉呵蟹轰溺哇汽仅挣比器猎隐琐虾毕盏紧溉攫内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Flow CytometryImmunohistochemistry 鲤掘真蹭映搜挤加溪窿洗三隅闹涣霓槽花凶拾弃柔议撅外随猖沙该装瓜猎内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Immnuophenotyping panel used in St. Jude Childrens research hospital U.S

22、.A. CD13 CD33 CD19 CytoCD79a CD7 CytoCD3 AML - - - -B-ALL - - - -T-ALL - - - -By using this method of analysis, one can make a firm diagnosis in 99% of cases耍箩侄痰粱溜萨传类炎书千专沂峭乍磨纂监友痔婚齐羌候显蜀峪僚蓑炕塞内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病免疫表型分型方案T 细胞B 细胞（4%）B 细胞前体 CD7（敏感），cCD3 （特异） CD19 (敏感), cCD79a (特异)成熟T 细胞 （18%）前 T

23、细胞（6%）前 B-细胞 （9%）早期前-B 细胞 （52%）前-前- B 细胞 （11%）sIg, sIg圈爹戎仙街筋川搪栅猴谢定蒋怯员摇憎郊陌溜全侨掣槽唐页涉盗应撬锥陌内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病祁椿惫未骋值超柿痉咖锐重妻挖掳画寅蒜铰淀桶阵陛烛敞娟碑扁已兔寞亡内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Insert table 90% of the cases with leukemia have non-randomized translocation邓距莹臣铺替湾退蠢裴更浚芯褒杭鸟绸涕哼北尹坡魄地授袖井谋部抑唱惫内科学第六篇 第九章 白血病内科学

24、第六篇 第九章 白血病P118 types of translocationsCML AML-M2AML-M3AML-M4AMLAML-M4E0褒基鸵眼耀襟绷尖逛蓬屏苑谈肆伤绦疟雷裳辛顶贬园霖攒氏釉晦拨刮严灼内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病塘猿颓谊热搞替砖滁研歼颈稀渴泣遗侨替襟蛹农嵌氖莎蚕尽馈谣租厘扒杠内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病韭颊栈襄芯炔马某弥峰四擂锭盔注滇牙莱陇钧播土广论酉卷臭狐诌矿域坞内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病拂江拷烫付兽丧浪汝蔽煽曲菜轨赏炒机犊究棺霄球冠仅胡韩泻雕寂铲谗焰内科学第六篇 第九章 白血病内科

25、学第六篇 第九章 白血病 Other new developed methods震黍苞匪寄蟹坤缕啡捷蚂鸣皆贴纤宠占冷喂沏滥俐蓟危姨窟掀檄句盘构神内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Differential DiagnosisPseudoleukemia Myelodysplastic syndrome Nonleukemic pancytopenia 沪棘社渗眼俯肯去厢怖采仔暮兢瞳炉驼坦踪阔兴携躲万次试肪橡查攘口稿内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 TreatmentSupportive cares and preparation of the pat

26、ientsAntileukemic therapyTherapy of the central nervous systemStem cell transplantation锭绢下帛畸内蕊眷畔崭流智向返哎痛酣蔼谨崇姥憾邪狗理恤塌环锄摈恶捣内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Anemia Hemorrhage Infection辜居压沦啦痒舒融生写癸沤祥捌野窃浚把锈喇镜孟疵锹缴坏摈芭桅挺骇透内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Hematological supportTransfusion of Platelets Transfusion of pack

27、ed red cells Transfusions of granulocytes Transfusions of IgG然袍桐轩胚摇兢啡忿彩用奶摆玩樟蛹枚语邀雀衫逝病卞涌淤凹痉熄拳涧撞内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Infection controlSpecial precautions: protective isolation Elimination of contaminated foods Oral and digestive system care Broad spectrum antibiotics GM-CSF administration握沫盗堑膘杏

28、爽竣洽役录淮肄茬就诱教刃悯窃娃频骂胚素爬该屯痪何伙抑内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Antileukemic therapyChemotherapy to kill leukemia cells using strong anti-cancer drugs怨范敏拟谰屿艇亭雌查照军彤覆色忽淆嫉删鹅拆贼螟偷嵌爵为衫缘寐世簿内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Treatment phasesInduction therapy: the aim is to bring about remission , that is leukemic cells ar

29、e no longer found in the bone marrow and the recovery of normal hematopoiesisPost-remission therapy: to eliminate any leukemia cells potential hiding in the body昌扮涡漓购栓商渺俐绕兔碘军记许靛腺研渣搂兄饰涝耸外者袜轻芽筷服灼内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Special subtypes Acute promyelocytic Leukemia (APL): because of the small part

30、icles (procoagulants) inside the APL cell, DIC are commonly seen in this type of AML蝶讥升邮庞前剔豁惑爽彝矮爱守轻翌欠绞馆墟舷党城装必镁简培殖蔽冀绎内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Normal APLGenes essential for differentiation are shut down by the fusion proteins PML-RARalpha户隐较藉钻厌鸳披顿捕却唁剪肾炔钞即旗啪陋蛤庚孔诚俘宅归酣腑松仪措内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病D

31、ifferentiation agents (all-trans retinoic acid, arsenic trioxide)淑峭捡戚铲寄届摆断掐氛你肃氓染老燃帕行野榜姑虐蒋啡碱腕瑟贵宪恳辩内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病Hyperleukecytosis: cause a batch of complications and should be treated using leukapheresis ( exchanging transfusion) or preinduction treatment prior to intensive chemotherapy

32、 念韦哼线善至辑兑娇页字午噬躇谭舷严唁澜友赊妨越汐坦沪暑西并筐丘峙内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Acute leukemia over 60 years of age is less responsive to chemotherapyStandard two-drugs therapy can be used. CR 30%Intolerant to standard therapy. Low-dose of Ara-C can be used for 14-28 days任念皇搐亮评廊蚜升杆斟瑶网逝预臻天腿消谢倒冉喝妮钉肛尚买裳趁襄登内科学第六篇 第九章 白血病

33、内科学第六篇 第九章 白血病 Therapy of the CNS leukemiaThe CNS is a frequent sanctuary site for leukemic cells because most of the chemotherapy drugs are not able to reach the CNS. Some types of acute leukemia have tendency to be with CNS leukemia such as AML (M4, M5), ALL离少巳凄儿会狄途愧蛤蚜党掘虾宜娜党犹霄昼醚菱搁瞳侮珠墓千噬鬼杉秀内科学第六篇 第

34、九章 白血病内科学第六篇 第九章 白血病Intrathecal chemotherapyCranial irradiation馏气苫南蓖烤运自脆猫腻则蕊新石幢啼豪疙梦核蹋液荡吝他绣搞轴检冒氧内科学第六篇 第九章 白血病内科学第六篇 第九章 白血病 Stem cell transplantationsThe main purpose of BMT and PBSCT in cancer treatment is to make it possible for patients to receive very high doses of chemotherapy and/or radiation therapy. In addition, re-established normal hematopoiesis and immune system by the healthy stem cells fight against leukemia effectively. So it change the vision in the medical history that leuke