血液生理巫宏博義大醫(yī)院血液及腫瘤科血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理Thinkof「血液」「血管中流動的液體」在人類是紅色的溫暖的腥味的黏稠的容易凝固的血液生理「血液」觀念演變亞里斯多得(BC384~322)希臘智者「血管內(nèi)裝空氣」蓋侖羅馬神醫(yī)、BC二世紀潮水說主宰西方醫(yī)學一千八百年(至17世紀)「血管內(nèi)裝血液，血液如潮水，消失于人體四周」血液生理哈維(WilliamHarvey,1578~1657)1628A.D.血液循環(huán)說「動物心臟與血液運動的解剖研究」「心臟是個泵，不斷打出及收回血液」「血液從心臟流向動脈，然后從靜脈收回心臟」但血液如何從動脈流至靜脈？馬爾必基義、1661用顯微鏡發(fā)現(xiàn)了微血管，完成哈維的循環(huán)說血液生理循環(huán)系統(tǒng)(Circulation)血液循環(huán)心臟：泵動脈：把血打出心臟靜脈：把血收回心臟肺臟：氣體交換排泄器官：腎、汗腺淋巴循環(huán)組織液淋巴管靜脈血液生理循環(huán)系統(tǒng)(Circulation)動脈微血管靜脈組織淋巴管血液循環(huán)淋巴循環(huán)淋巴組織滲出肺CO2O2肺動脈肺靜脈排泄廢物養(yǎng)分、荷爾蒙LVLARARV血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理「血液」的來源&分布制造卵黃囊:胚胎2周后肝臟+脾臟:胚胎6周10周后骨髓:胚胎24周后成人四肢腸骨造血(20歲以前)分布骨髓循環(huán)系統(tǒng)脾臟、肝臟血液生理造血細胞Stemcell(干細胞):self-renewalTotipotent(全能)&pluripotent(多能)干細胞CD34+(1%ofBM;0.01%ofPB)PPSC(1%ofCD34+)Committed(委任)stemcellProgenitor祖先細胞:mitosis&maturationPrecursor前軀細胞:maturation血液生理血液組成血液占體重7%3500cc/50Kg;5000cc/70kg.細胞45%volume紅血球數(shù)~5,000K/ul(95%)白血球數(shù)~5K/ul(0.1%)血小板數(shù)~250K/ul(5%)血漿55%volume血液生理血球家譜StemCellMyeloidStemcellLymphoidStemcellRBC紅血球Neutrophil中性球Monocyte單核球Eosinophil伊紅球Basophil嗜鹼球Platelet血小板T/NKT細胞stemcellNK細胞

B細胞Plasma細胞LNTHYMUSBM血液生理血液生理血球數(shù)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&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理紅血球雙凹盤Biconcave,8*8*2m,體積90fLNonucleus,nomitochondria.RBC:500萬/ulReticulocyte(網(wǎng)狀球):1萬，含Golgi,mitochondriaCytoplasmHemoglobin(Hb)血紅素Themajorprotein(34%ofRBC胞質(zhì))Life:100-120dmacrophage吞食.Energy:glucose2,3-DPG,ATPNADPH下降老化血液生理RBCSeriesPronormoblastBasophilicnormoblastPolychromaphilicOrthrochromicReticulocyte,RBCBMPB(1day)血液生理異常紅血球形態(tài)鐮刀紅血球破碎紅血球球形紅血球靶狀紅血球咬破紅血球水滴紅血球血液生理紅血球功能從肺取得O2=動脈氧Hb(15g/100cc)x1.34(cc/g)~20cc/100cc釋出給組織=動脈氧-靜脈氧氧氣解離曲線(O2dissociationcurve)(Hb)氧結(jié)合力越弱，組織得氧越多如：H+,DPG,temp增加從組織移除CO2血液生理氧氣供應(O2delivery)O2delivery=CO*Hb*1.34*SaO2CO(L/min):cardiacoutput心血輸出量Hb(g/dL):hemoglobin血紅素厚度1.34(mL/g):O2/Hb血紅素飽和時的攜氧量SaO2(%):動脈血紅素的氧氣飽和度血液生理Erythropoietin(造血)EPO紅血球生成素34Kd醣蛋白Kidney:80-90%Liver:10-20%組織中O2不足ArteryO2(腎)AnemiaPoorbloodflowLowbloodvolume血液生理紅血球疾病紅血球減少:貧血癥狀：蒼白、疲倦紅血球增多癥癥狀：面紅、頭痛血液生理血液生理貧血(anemia)定義：紅血球(血紅素)不足。Hbg/dLHb<13.5(male)or<12(female)RBC#(/uL)~Hb/3Hct(hematocrit,血球容積%)~Hb*3貧血代償compensation骨髓加班:acute~3folds;chronic~5-8foldsO2DC(氧氣解離曲線)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,電廠,喘,發(fā)燒Physicalexamination臉(chipmunk),眼,舌,nail,LN,L/S,DREBloodroutine血液常規(guī)PBsmear血抹片Reticulocyte網(wǎng)狀球血液生理RedCellIndexRBC#(M/ul=M/cumm)紅血球數(shù)Hb(g/dl)血紅素Hct(%)=MCVxRBCx0.1(~Hbx3)血容MCV(fl=u3)=Hct/RBCx10血球容積血液生理Anemia:MCV分類法Microcytic小球:Hb缺乏deccytoplasmIDA,thalassemiaMacrocytic大球:Nu分化障礙,reticulocyteMegaloblastic,hemolysis,aplasticanemiaNormocytic正球:stemcell(Nu,Hb異常)Hemolysis,acutebloodloss,aplasticanemiamicrocyticmacrocyticnormocytic血液生理IDA&BloodLossThemostcommoncauseofanemia.Acutebloodloss:trauma,GI,MCIrondeficiencyanemia缺鐵性貧血Chronicbloodloss:MC,腸胃Diet,diarrhea血液生理Hemolyticanemia

溶血性貧血(1)G6PD(葡萄糖6-磷酸去氫酶)deficiency最常見的酵素缺乏癥發(fā)燒,蠶豆,藥品氧化傷害溶血藥物：阿斯匹靈aspirin,瘧疾藥,磺ㄢ藥,氯霉素,維持生活素C,K血液生理Hemolyticanemia

溶血性貧血(2)Thalassemia(海洋性貧血)alfa型4%,beta型2%inTaiwandecreaseglobinabimbalanceHAdecreaseMCV&HbAincreaseHbA2(a2d2),HbF(a2g2),targetcells,N-RBCtransfusion,排鐵,篩檢Sicklecellanemia黑人(8%)，臺灣罕見HbS(b6gluval)sickling(whenhypoxemia)splenichemolysis,血管阻塞自體脾切除血液生理巨核性貧血MegaloblasticAnemia原因：B12,葉酸DNA合成核成熟,分裂缺乏核成熟,分裂遲緩；但RNA正常變大飲食缺乏:alcohol,素食,早產(chǎn),TPN吸收不良(葉酸在空腸,B12藉內(nèi)在因子在回腸)perniciousa.(惡性貧血),胃切除,小腸疾病消耗增加:懷孕,溶血診斷：血球變大(減少)、B12、葉酸降低治療：補充B12、葉酸血液生理AplasticAnemia再生不良性貧血原因:80%不明骨髓造血功能不良AnemiaLeukopeniaThrombocytopenia血液生理紅血球增多癥相對性:水分減少造成絕對性:紅血球增多造成真性:即「原發(fā)性」骨髓增生造成Polycythemiavera黏稠度=10xwater微血管阻塞續(xù)發(fā)性：Secondarypolycythemia高地(4km)、吸煙、心肺疾病tissuehypoxia腎臟、肝臟癌癥EPOactivity血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理白血球─免疫功能顆粒球(granulocyte,PMN) 血中中性球neutrophil60%─細菌、霉菌 6h依紅球eosinophil3%─寄生蟲、過敏嗜鹼球basophil1%─過敏、抗凝肥胖細胞mastcell nil淋巴球(lymphocyte)30% mo-yrsBcell(15%)─體液免疫antibodyTcell(70%)─細胞免疫、抗癌NaturalKillercell(15%)─抗癌單核球(monocyte)6%─促進免疫 15h巨噬球、組織球─吞噬細菌、霉菌血液生理PMNSeriesMyeloblastSegmentformBandformMetamyelocyteMyelocytePromyelocyte骨髓母細胞前骨髓細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單核母細胞前單核細胞巨噬細胞/組織巨噬細胞單核細胞血液生理白血球疾病數(shù)量改變(7000+-3500/ul)增多：發(fā)炎、感染、過敏減少：制造不足、消耗太快、脾臟腫大結(jié)構(gòu)異常白血病(血癌)急性、慢性骨髓性、淋巴性骨髓瘤、淋巴瘤表現(xiàn)：貧血、發(fā)燒、出血、肝脾腫大血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理血小板2-3u,負責血液凝固無細胞核,但含有許多與發(fā)炎,及血液凝固有關(guān)之化學物質(zhì)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巨核母細胞巨核球前巨核球血液生理血小板疾病血小板減少因制造不足、消耗太快、稀釋、脾臟腫(表12-5)血小板異常NSAID(non-steroidanti-inflammatorydrug)aspirin血小板增多血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理循環(huán)系統(tǒng)(Circulation)動脈微血管靜脈組織淋巴管血液循環(huán)淋巴循環(huán)淋巴組織滲出肺CO2O2肺動脈肺靜脈排泄廢物養(yǎng)分、荷爾蒙LVLARARV血液生理淋巴系統(tǒng)血液生理淋巴循環(huán)淋巴組織Primary原發(fā)性:淋巴球生長骨髓、胸腺。Secondary次發(fā)性:淋巴球聚集淋巴腺、脾、黏膜淋巴組織(MALT)淋巴管、淋巴總管、胸管(thoracicduct)內(nèi)容物淋巴液組織液淋巴球：B、T、NK血液生理1o淋巴組織：骨髓、胸腺BNKT血液生理2o淋巴組織：淋巴腺、脾臟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.減少(<1500/ul)Dec.production:advcancersorHodgkindisease,AA,congenitalIgdeficiencyInc.destruction:steroid,cytotoxictherapy過度流失：胸管、腸道腫瘤、右心衰竭AIDS、腎衰竭、重癥肌無力、miliaryTB、SLE血液生理惡性淋巴球疾病淋巴性白血病急性,慢性惡性淋巴瘤：淋巴腺腫大何杰金氏非何杰金氏漿細胞腫瘤單發(fā)性、多發(fā)性骨髓瘤血液生理Immunity免疫反應先天innateimmunityWBC,tissuemacrophage胃酸、消化酶、SkinLysozyme,鹼性蛋白,complement,NKcells后天acquiredimmunity:lymphocyteCellular:Tcell Humoral:BcellAb--antigen(>8Kd)Hapten輔抗原(<8Kd)+proteinAg血液生理Inflammation(發(fā)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血液生理腫瘤的免疫反應

AgNon-selfMHC1SelfMHC1Ab

ComplementlysisADCC(NK,PMN,Ma)APCMoMHC2B7/ICAMTh2Th1CTLNKIFN,IL2B血液生理StressandImmune血液生理Outlines血液&循環(huán)血液的產(chǎn)生&分布紅血球&疾病白血球&疾病血小板&疾病淋巴、免疫&疾病輸血醫(yī)學血液生理血型(紅血球)血型超過300種胞膜之蛋白質(zhì)或醣化脂質(zhì)glycolipid重要抗原：ABO(醣化脂質(zhì))&Rh(蛋白質(zhì))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