血液生理课件

血液生理巫宏博义大医院血液及肿瘤科血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理Thinkof「血液」「血管中流动的液体」在人类是红色的温暖的腥味的黏稠的容易凝固的血液生理「血液」观念演变亚里斯多得(BC384~322)希腊智者「血管内装空气」盖仑罗马神医、BC二世纪潮水说主宰西方医学一千八百年(至17世纪)「血管内装血液，血液如潮水，消失于人体四周」血液生理哈维(WilliamHarvey,1578~1657)1628A.D.血液循环说「动物心脏与血液运动的解剖研究」「心脏是个泵，不断打出及收回血液」「血液从心脏流向动脉，然后从静脉收回心脏」但血液如何从动脉流至静脉？马尔必基义、1661用显微镜发现了微血管，完成哈维的循环说血液生理循环系统(Circulation)血液循环心脏：泵动脉：把血打出心脏静脉：把血收回心脏肺脏：气体交换排泄器官：肾、汗腺淋巴循环组织液淋巴管静脉血液生理循环系统(Circulation)动脉微血管静脉组织淋巴管血液循环淋巴循环淋巴组织渗出肺CO2O2肺动脉肺静脉排泄废物养分、荷尔蒙LVLARARV血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理「血液」的来源&分布制造卵黄囊:胚胎2周后肝脏+脾脏:胚胎6周10周后骨髓:胚胎24周后成人四肢肠骨造血(20岁以前)分布骨髓循环系统脾脏、肝脏血液生理造血细胞Stemcell(干细胞):self-renewalTotipotent(全能)&pluripotent(多能)干细胞CD34+(1%ofBM;0.01%ofPB)PPSC(1%ofCD34+)Committed(委任)stemcellProgenitor祖先细胞:mitosis&maturationPrecursor前躯细胞:maturation血液生理血液组成血液占体重7%3500cc/50Kg;5000cc/70kg.细胞45%volume红血球数~5,000K/ul(95%)白血球数~5K/ul(0.1%)血小板数~250K/ul(5%)血浆55%volume血液生理血球家谱StemCellMyeloidStemcellLymphoidStemcellRBC红血球Neutrophil中性球Monocyte单核球Eosinophil伊红球Basophil嗜硷球Platelet血小板T/NKT细胞stemcellNK细胞

B细胞Plasma细胞LNTHYMUSBM血液生理血液生理血球数目分布比率PB(血液)BM(骨髓)RBC95%20%WBC0.1%79%Neutrophil60%Myeloid62%Eosinophil3%Basophil1%Monocyte6%Lymphocyte30%17%Platelet5%1%血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理红血球双凹盘Biconcave,8*8*2m,体积90fLNonucleus,nomitochondria.RBC:500万/ulReticulocyte(网状球):1万，含Golgi,mitochondriaCytoplasmHemoglobin(Hb)血红素Themajorprotein(34%ofRBC胞质)Life:100-120dmacrophage吞食.Energy:glucose2,3-DPG,ATPNADPH下降老化血液生理RBCSeriesPronormoblastBasophilicnormoblastPolychromaphilicOrthrochromicReticulocyte,RBCBMPB(1day)血液生理异常红血球形态镰刀红血球破碎红血球球形红血球靶状红血球咬破红血球水滴红血球血液生理红血球功能从肺取得O2=动脉氧Hb(15g/100cc)x1.34(cc/g)~20cc/100cc释出给组织=动脉氧-静脉氧氧气解离曲线(O2dissociationcurve)(Hb)氧结合力越弱，组织得氧越多如：H+,DPG,temp增加从组织移除CO2血液生理氧气供应(O2delivery)O2delivery=CO*Hb*1.34*SaO2CO(L/min):cardiacoutput心血输出量Hb(g/dL):hemoglobin血红素厚度1.34(mL/g):O2/Hb血红素饱和时的携氧量SaO2(%):动脉血红素的氧气饱和度血液生理Erythropoietin(造血)EPO红血球生成素34Kd醣蛋白Kidney:80-90%Liver:10-20%组织中O2不足ArteryO2(肾)AnemiaPoorbloodflowLowbloodvolume血液生理红血球疾病红血球减少:贫血症状：苍白、疲倦红血球增多症症状：面红、头痛血液生理血液生理贫血(anemia)定义：红血球(血红素)不足。Hbg/dLHb<13.5(male)or<12(female)RBC#(/uL)~Hb/3Hct(hematocrit,血球容积%)~Hb*3贫血代偿compensation骨髓加班:acute~3folds;chronic~5-8foldsO2DC(氧气解离曲线)血液生理NormalHb&MCVAge&SexHb(g/dL)MCVNewborn19-17119-1111m~2m/o13-111003m~3y/o1188Adolescence1388Adultman1588Adultwoman1388MPwoman1488Pregnant1290血液生理DiagnosisofAnemia

ImportantToolsHistoryFH,diet,排泄,MC,Pb,苯,电厂,喘,发烧Physicalexamination脸(chipmunk),眼,舌,nail,LN,L/S,DREBloodroutine血液常规PBsmear血抹片Reticulocyte网状球血液生理RedCellIndexRBC#(M/ul=M/cumm)红血球数Hb(g/dl)血红素Hct(%)=MCVxRBCx0.1(~Hbx3)血容MCV(fl=u3)=Hct/RBCx10血球容积血液生理Anemia:MCV分类法Microcytic小球:Hb缺乏deccytoplasmIDA,thalassemiaMacrocytic大球:Nu分化障碍,reticulocyteMegaloblastic,hemolysis,aplasticanemiaNormocytic正球:stemcell(Nu,Hb异常)Hemolysis,acutebloodloss,aplasticanemiamicrocyticmacrocyticnormocytic血液生理IDA&BloodLossThemostcommoncauseofanemia.Acutebloodloss:trauma,GI,MCIrondeficiencyanemia缺铁性贫血Chronicbloodloss:MC,肠胃Diet,diarrhea血液生理Hemolyticanemia

溶血性贫血(1)G6PD(葡萄糖6-磷酸去氢酶)deficiency最常见的酵素缺乏症发烧,蚕豆,药品氧化伤害溶血药物：阿斯匹灵aspirin,疟疾药,磺ㄢ药,氯霉素,维持生活素C,K血液生理Hemolyticanemia

溶血性贫血(2)Thalassemia(海洋性贫血)alfa型4%,beta型2%inTaiwandecreaseglobinabimbalanceHAdecreaseMCV&HbAincreaseHbA2(a2d2),HbF(a2g2),targetcells,N-RBCtransfusion,排铁,筛检Sicklecellanemia黑人(8%)，台湾罕见HbS(b6gluval)sickling(whenhypoxemia)splenichemolysis,血管阻塞自体脾切除血液生理巨核性贫血MegaloblasticAnemia原因：B12,叶酸DNA合成核成熟,分裂缺乏核成熟,分裂迟缓；但RNA正常变大饮食缺乏:alcohol,素食,早产,TPN吸收不良(叶酸在空肠,B12藉内在因子在回肠)perniciousa.(恶性贫血),胃切除,小肠疾病消耗增加:怀孕,溶血诊断：血球变大(减少)、B12、叶酸降低治疗：补充B12、叶酸血液生理AplasticAnemia再生不良性贫血原因:80%不明骨髓造血功能不良AnemiaLeukopeniaThrombocytopenia血液生理红血球增多症相对性:水分减少造成绝对性:红血球增多造成真性:即「原发性」骨髓增生造成Polycythemiavera黏稠度=10xwater微血管阻塞续发性：Secondarypolycythemia高地(4km)、吸烟、心肺疾病tissuehypoxia肾脏、肝脏癌症EPOactivity血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理白血球─免疫功能颗粒球(granulocyte,PMN) 血中中性球neutrophil60%─细菌、霉菌 6h依红球eosinophil3%─寄生虫、过敏嗜硷球basophil1%─过敏、抗凝肥胖细胞mastcell nil淋巴球(lymphocyte)30% mo-yrsBcell(15%)─体液免疫antibodyTcell(70%)─细胞免疫、抗癌NaturalKillercell(15%)─抗癌单核球(monocyte)6%─促进免疫 15h巨噬球、组织球─吞噬细菌、霉菌血液生理PMNSeriesMyeloblastSegmentformBandformMetamyelocyteMyelocytePromyelocyte骨髓母细胞前骨髓细胞血液生理淋巴球的生成TdT+CD34TdT+CD34TdT+CD7T8T4TdT+DRCD34TdT+DRCD34TdT+DRCD10TdT+DRCD10DRCD21CD22PlasmacellTdT+CD7CD19CD19CD20CD19CD19CD20CD2CD2CD2CD2sIgCD2CD7CD5CD5CD5CD3CD3CD4CD4CD8CD8CD1CD1CuPrecursorBEarlyThymocyteCommonMaturePre-BBPlasmacyteBT血液生理MonocyteSeriesMonoblastMonocytePromonocyteMacrophage/Histiocyte单核母细胞前单核细胞巨噬细胞/组织巨噬细胞单核细胞血液生理白血球疾病数量改变(7000+-3500/ul)增多：发炎、感染、过敏减少：制造不足、消耗太快、脾脏肿大结构异常白血病(血癌)急性、慢性骨髓性、淋巴性骨髓瘤、淋巴瘤表现：贫血、发烧、出血、肝脾肿大血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理血小板2-3u,负责血液凝固无细胞核,但含有许多与发炎,及血液凝固有关之化学物质血液生理CLOTHemostasissystemPLATELETFIBRINOLYSISENDOTHELIUMCOAGULATIONFibrinPlasminThrombinPlugTm,PL,VWFvesselorcellinjuryTFPI,AT3,PC/PSVASOCONSTRICTION血液生理HemostasisSystem血液生理CoagulationCascade血液生理PlateletSeriesMegakaryoblastMegakaryocytePromegakaryocytePlatelet巨核母细胞巨核球前巨核球血液生理血小板疾病血小板减少因制造不足、消耗太快、稀释、脾脏肿(表12-5)血小板异常NSAID(non-steroidanti-inflammatorydrug)aspirin血小板增多血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理循环系统(Circulation)动脉微血管静脉组织淋巴管血液循环淋巴循环淋巴组织渗出肺CO2O2肺动脉肺静脉排泄废物养分、荷尔蒙LVLARARV血液生理淋巴系统血液生理淋巴循环淋巴组织Primary原发性:淋巴球生长骨髓、胸腺。Secondary次发性:淋巴球聚集淋巴腺、脾、黏膜淋巴组织(MALT)淋巴管、淋巴总管、胸管(thoracicduct)内容物淋巴液组织液淋巴球：B、T、NK血液生理1o淋巴组织：骨髓、胸腺BNKT血液生理2o淋巴组织：淋巴腺、脾脏TRCMaDCBT血液生理SuperficialLymphNodes血液生理良性淋巴球疾病增加(>5000/ul)Virus,TB,syphilis,pertussis,stress.减少(<1500/ul)Dec.production:advcancersorHodgkindisease,AA,congenitalIgdeficiencyInc.destruction:steroid,cytotoxictherapy过度流失：胸管、肠道肿瘤、右心衰竭AIDS、肾衰竭、重症肌无力、miliaryTB、SLE血液生理恶性淋巴球疾病淋巴性白血病急性,慢性恶性淋巴瘤：淋巴腺肿大何杰金氏非何杰金氏浆细胞肿瘤单发性、多发性骨髓瘤血液生理Immunity免疫反应先天innateimmunityWBC,tissuemacrophage胃酸、消化酶、SkinLysozyme,硷性蛋白,complement,NKcells后天acquiredimmunity:lymphocyteCellular:Tcell Humoral:BcellAb--antigen(>8Kd)Hapten辅抗原(<8Kd)+proteinAg血液生理Inflammation(发炎)Vasodilatation,increasepermeability,coagulation,WBCinfiltrate,cellswellingLocaltissuemacrophage:in1hrBloodneutrophilinfiltration:inhrsBloodmonocyte-macrophage:after8hrsBMmyeloidhyperplasia:after3-4days血液生理Phagocytosis血液生理ImmuneInteractionsNKmBAPCCTLmTTh血液生理AntibodyIgG:75%;secondaryresponseIgA:mucosalprotectionIgM:primaryresponseIgD:?IgE:allergymast,basophilHCLCvariable血液生理肿瘤的免疫反应

AgNon-selfMHC1SelfMHC1Ab

ComplementlysisADCC(NK,PMN,Ma)APCMoMHC2B7/ICAMTh2Th1CTLNKIFN,IL2B血液生理StressandImmune血液生理Outlines血液&循环血液的产生&分布红血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病输血医学血液生理血型(红血球)血型超过300种胞膜之蛋白质或醣化脂质glycolipid重要抗原：ABO(醣化脂质)&Rh(蛋白质)血液生理BloodComponents血液生理BloodComponentsWholeblood(WB)RBC:packedredbloodcell(PRBC)Leukocyte-reducedRBC3rdgeneration(adhesion)filterremove99.9%WBC.WashedRBCplasmawashedoffbyNS.Frozen,deglycerolizedRBCupto10y(65C),IrradiatedRBC25Gyx4w.PlateletsRDP(randomdonorplatelet):plateletconcentrate(PC)SDP(singledonorplatelets):apheresis(PH)血液生理PlasmaComponentsFFP,plasmaderivedvolumeexpanders(albumin)CoagulantsCryoprecipitateF8concentratesF9concentratesAT3concentratesRecombinantF7a,FibrinSealantsAbImmuneGlobulins,RhIG血液生理AcutetransfusionreactionsAcutehemolytictransfusionreactionsFebrilenonhemolyticreactionsTransfusion-relatedacutelunginjury(ARDS)Allergicreaction,anaphylaxisHy