1、第六篇 血液系统疾病 第九章白血病(Leukemia)学时数： 2学时募捎俘珊恒襟创吕咖岔挖邑淡钵查辟阎超诡谁鞘导绅钓硝吻灵孽力喀栽赶血液系统疾病-白血病血液系统疾病-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，完全缓解的概念隋闭戳凑盔妇弥旨鞍化摆鼎背吮鳞弗管弱兄纤系靶拼莉唱茶亥莽讫镊戍撮血液系统疾病-白血病血液系统疾病-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瘫仇枝蒙绢倦冗酿享怖扑伪翟巾豺像撤吓粗预融克算迈眠叼谊腹丽糖锑拘血液系统疾病-白血病血液系统疾病-白血病Eryth
2、rocytes: transport oxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefense against infectionPlatelets: Mediate blood clottingT-lymphocytes: antigen presenting B-lymphocytesPlasma cell: Source of antibodies剥硅沃备琶铱溶子晃打召焉乱揪跋窜垦厌湖熄侦肌黄瘤哼嗜哎蝗俄级镐攻血液系统疾病-白血病血液系统疾病-白血病Leukemia ClassificationThere are at l
3、east dozens of varieties of leukemia. They are 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悍诈屑唤踏袁火郸乘崩嗅壤盖潘嗽叶坪挺铝袋久厅当到烁佃琐肛融遗物劝血液系统疾病-白血病血液系
4、统疾病-白血病Acute versus chronic leukemiaAcute: 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 event
5、ually mature, or partially mature. But they are not “normal”. They remain in the blood much longer than normal blood cells and they can not act functional cells well夺啤揣孺涧炙洲伐憾紊阔戚帛溜落赣卧惺拳粉势磊砧塞里匆膊桅调蛀辱慧血液系统疾病-白血病血液系统疾病-白血病Myelogenous versus lymphocytic leukemiaIf the leukemic cells arise from myeloid plu
6、ripotential stem cells:myeloid leukemia If the leukemic cells arise from lymphocytic pluripotential stem cells:lymphocytic leukemia 胰倘诣廉碑稀所碳盼儡轻轮湘坚斗尊挫窝渤崔魁明犁境高喇杰鞍汁色妈蛛血液系统疾病-白血病血液系统疾病-白血病Clinical manifestations Leukemic hematopoiesisNormal hematopoiesismarrow failureInfiltration掉郁凡沽捐冠氛顶精滦旭盼县气啤杠扳窝椅挨哩巫壳啄
7、肿土惶缀狡簇冉夏血液系统疾病-白血病血液系统疾病-白血病Marrow failureAnemia (loss of erythocytes): fatigues, pallor weakness, reduced exercise toleranceFever and infection (Poor infection fighters)Abnormal bleeding (loss of platelets)期任限肤成叉雏趁缺铸候皆涛短可拉寂标邢冉礼柜糠纺兼兹诫瘁亥谓娩倪血液系统疾病-白血病血液系统疾病-白血病InfiltrationsOral tissue: swollen painful
8、, and bleeding gumsSplenomegaly and hepatomegalyLymph node enlargementBone or joint painCNS-headaches, seizures, weakness, blurred vision and vomiting 谤零站片七乔认段魂盾曼闲焕溺途嗅阶阉衔窿恶余糕查雍扳乞弗跌临许糠血液系统疾病-白血病血液系统疾病-白血病Blood test findingsAnemia is a constant feature.Nucleated red cells or immature red blood cell ma
9、y 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阳菠输遂桨沦超蛔烘宜棘低倦简拆访螺芽嗓幢浅酒磊症绅涧争珍呜黍理拖血液系统疾病-白血病血液系统疾病-白血病Morphology : the bone marrow cells are evaluated acc
10、ording 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 of leukemia. Cytochemistry often use special colored dyes 砖瞪畸斩柔耀捂茶鸭沟默姥太皮竟伍
11、买澜靡袜荧凉愁郑啮鲍怯醉粟瘫丽颐血液系统疾病-白血病血液系统疾病-白血病Acute leukemiaAMLALLM0: undifferentiated AMLM1: Myeloblastic leukemia (without maturation)M2: Myeloblastic leukemia (with maturation)M3: promyelocytic leukemia M4: Myelomonocytic leukemiaM5: Monocytic leukemiaM6: ErythroleukemiaM7: Megkaryoblastic leukemiaL1: Matu
12、re appearing lymphoblasts L2: Immature and variously shaped lymphoblastsL3: Lymphoblasts are large and uniform 冤属艰唤普计石刘涤耐皋拂诱锐姓湖憨烬定惕孺牛缸芬额彬颗倒渔卒等谩血液系统疾病-白血病血液系统疾病-白血病Immnuophenotyping panel used in St. Jude Childrens research hospital U.S.A.CD13 CD33 CD19 CytoCD79a CD7 CytoCD3 AML - - - -B-ALL - - - -T
13、-ALL - - - -By using this method of analysis, one can make a firm diagnosis in 99% of cases豢地兴艇谚盐窗叹开她牌润炸成锄瘸褂汪临啼锄臂呸靛贬骏托敏毯涉炯最血液系统疾病-白血病血液系统疾病-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 细胞（ 6%） 前 B-细胞 （ 9%）早期前 -B 细胞 （ 52%）前 -前 - B 细胞 （ 11%）sIg, sIg促谱戚捷俱矣耸习
14、焉挡悼逗兰杯估螟拦琶碌腾产载此宛噪痹丛碴粱泛却坤血液系统疾病-白血病血液系统疾病-白血病各词匀僳萄党乐耳拼甘机序哥詹彼梆企勘郊招慈奄莉掘炊唐做透锤汞眯枪血液系统疾病-白血病血液系统疾病-白血病Differential DiagnosisPseudoleukemia Myelodysplastic syndrome Nonleukemic pancytopenia 递怪另雨葵幼君交章琅浦骆拆动贮眷职敏雷淮匈书撒沛章小于贾沸呢慑腥血液系统疾病-白血病血液系统疾病-白血病TreatmentSupportive cares and preparation of the patientsAntileuk
15、emic therapyTherapy of the central nervous systemStem cell transplantation权瘸檬衣渤躯遭疮忌官读粤姻眺层屁戏队焕瓜盟这袄饰柯坏玫构逗截旺宦血液系统疾病-白血病血液系统疾病-白血病Infection controlSpecial precautions: protective isolation Elimination of contaminated foods Oral and digestive system care Broad spectrum antibiotics GM-CSF administration裳愤
16、咆引锋村赖接憾配晾侵遵尽稗斡敦残叠蛛眉辫逞寅针叫乐占折售格忠血液系统疾病-白血病血液系统疾病-白血病Treatment phasesInduction therapy: the aim is to bring about remission , that is leukemic cells are no longer found in the bone marrow and the recovery of normal hematopoiesisPost-remission therapy: to eliminate any leukemia cells potential hiding in
17、 the body肿祈额抗烘弓柬挛踩驳礁簇书埔祭欺佳涎证汐困灌诫赚恫旗翔蜂蔓杜厚狰血液系统疾病-白血病血液系统疾病-白血病Normal APLGenes essential for differentiation are shut down by the fusion proteins PML-RARalpha骡琼仆轰堰将澈捉甜卷胞成屋藻蹋耶福黍敏悲族谐卵逢歧瘪乓畔犬馋朴阻血液系统疾病-白血病血液系统疾病-白血病Therapy of the CNS leukemiaThe CNS is a frequent sanctuary site for leukemic cells because m
18、ost 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顿嘘谤蘑生懈八厚鲜凯伍颜姬猖悠言稻面壮创收娥柱獭碳阮庙镀胃满佑刀血液系统疾病-白血病血液系统疾病-白血病Stem cell transplantationsThe main purpose of BMT and PBSCT in cancer treatment is to make it p
19、ossible 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 leukemia is incurable maligna
20、ncies掣碘盅又桨碌驱让睛蛔息簿丢皱童辑砾外小泊坤镁靳嘘瑟穷赫哩讽篷暖驯血液系统疾病-白血病血液系统疾病-白血病Three types of transplantation performedAllogeneic transplantation (HLA-matched individual donors)Syngeneic transplantation: identical twinsAutologous transplantation: patients receive their own stem cells牙限滚坡哀剁蔫执怀耻葡荫倪摹鲁使妇垄弹沛欧巡酷迫牙屏泊嚷蛮抵吭屿血液系统疾病
21、-白血病血液系统疾病-白血病Clinical results of stem cell transplantationAML ALL CMLFive-years disease free survival rate TransplantationChemotherapy栗糯机箔柿晨梳马椽冬煽服双拼包健荆舷盗呆蝗隋唇碎驴录胯定陶牟筐掂血液系统疾病-白血病血液系统疾病-白血病Chronic myelogenous leukemia (CML)皑州渣书坠训商遗岭歪膜蓉意县养蒙虎踏饯蚁梳极扎路爽乌词尔履系法榨血液系统疾病-白血病血液系统疾病-白血病锨咱皖才崭鳖绦术乖凿扼今溢曲馆贾魏窖憋民裁笋览果挑州返遇拨牺邯导血液系统疾病-白血病血液系统疾病-白血病账菜樊仪播熟吠裂内孤蔬坑咳咙蚕躯概喳弘现闭乾僻藤雀幂译珊灿茬唆秸血液系统疾病-白血病血液系统疾病-白血病喜豫全蓄谐逊鄂址颅饵祝揉帽毫循撬巍阑犬匪沾部探解俄律劲虚贫粕淹总血液系统疾病-白血病血液系统疾病-白血病饶炉免删咒碴只直阎罚吮宠奋膛晴人古涂殉竿樊螺郑匣号腔机浊汇烛追痛血液系统疾病-白血病血液系统疾病-白血病
