易栓症神内病例ppt课件

易栓症,1,Normalhemostasis（止血）,adhesion粘附,aggregation聚集,coagulationpathway凝血途径,2,3,Coagulationbalance,anticoagulantmechanisms,procoagulantmechanisms,fibrinformation,4,Fibrinmonomer,Endothelialcellsurface,5,coagulationfactorsanticoagulantproteinsgain-of-functionmutationsfibrinolysisantiphospholipidAb,fibrinformation,anticoagulantmechanisms,procoagulantmechanisms,6,易栓症（Thrombophlilia）,指由于抗凝蛋白、凝血因子、纤溶蛋白等的遗传性或获得性缺陷，或存在获得性危险因素，而容易发生血栓栓塞的疾病状态。不是单一的疾病。血栓栓塞类型：静脉血栓栓塞,7,AnnualincidenceofDVT,Whites0.8-1.2x1000HongKongChinese0.16x1000Liuetal,HongKongMedJ2002,rateratio(vswhite)AfricanAmericans1.27(1.07-1.51)Hispanic0.60(0.54-0.67)Asians/PacificIslanders0.26(0.22-0.30)*Whiteetal,AnnInternMed1998,*idiopathicorsecondary,8,VTE:amultifactorialdisease,acquired,genetic,mixed,transient,VTE,riskfactors,9,theoldstory.,deficiencyofanticoagulantproteinsyearofdiscoveryantithrombin1965proteinC1981proteinS1984,10,ANTITHROMBIN,PROTEINC,PROTEINS:typeofdeficiency,TypeI:Quantitativedeficiency,TypeII:Qualitativedeficiency,antigenictest,functionaltest,functionaltest,=antigenictest,11,Prevalenceofdeficiencies,generalunselectedpopulationVTEpatientsantithrombin0.02-0.2%1%proteinC0.1-0.5%3%proteinS?1-2%,12,RiskofVTEassociatedwithdeficiencies,increaserelativeriskantithrombin5-50proteinC7-15proteinS6-10,13,yearofdiscoveryresistancetoactivatedproteinC1993/94andfactorVLeiden(G1691A)prothrombinmutation(G20210A)1996,14,theoldstory.,olderagecancerantiphospholipidantibodiespreviousvenousthromboembolism,15,AgeandVTE,0-9,10-19,20-29,30-39,40-49,50-59,60-69,70-79,79,600,500,400,300,200,100,0,Andersonetal,1991,IncidenceRateper100,000,Females,Males,16,CancerandVTE,Tissuefactor,constitutiveofmalignantcells,promotesangiogenesisandincreasesplasminproduction(metastases)Prothromboticcancersubstances(cancerprocoagulant,IL1,TNF,etc.)TheriskofVTEishigherifchemotherapy(tamoxifen,thalidomide,L-asparaginase)VTEandoccultcancer,17,yearofdiscoveryhyperhomocysteinemia1994highfactorVIII1995,18,theoldstory.,surgeryandmajortraumaprolongedimmobilizationpregnancy/puerperium(6weekspostpartum)oralcontraceptives/hormonereplacementtherapy,19,yearofdiscoveryActivatedproteinCresistance1993Airtravel1999,20,21,F：familyhistory（尤其是强家族史，即家族中有至少两例同类型血栓患者）U：unusuallocationsofthrombosis（腹腔、颅内等部位）R：recurrentepisodesofthrombosisY：year（45y）符合上述任1条均建议筛查易栓症指标,FURY,22,易栓症初筛项目PT、aPTT、Fib、(TT?)抗磷脂抗体(LA、ACA)空腹同型半胱氨酸FVIII:C蛋白C活性蛋白S活性抗凝血酶活性APC-RFVLeiden凝血酶原G20210A,国内大多三级医院可以检测,国内部分三级医院开展检测,国外部分医院开展,23,不应在血栓急性期筛查易栓症的理由1.检测结果不影响急性期的初始治疗（除非APTT延长，考虑存在狼疮抗凝物）；2.急性期可因轻度消耗或急性炎症，引起几种凝血成分的一过性降低或升高，包括PC、PS、AT和多种凝血因子。,24,分析易栓症筛查结果时需防范以下误区：检测结果/报告有误;接受维生素K拮抗剂的患者或者维生素K缺乏症患者诊断PC或PS缺乏时;接受维生素K拮抗剂(可增加AT浓度)的患者排除AT缺乏时;仅凭一次检测结果诊断PC、PS或AT缺乏;急性VT或其他疾病时取血诊断或排除任何一种易栓症;仅凭一次检测结果诊断抗磷脂抗体综合征;检测项目选用不当。,25,易栓症延长疗程抗凝的指征一种以上危险因素(不包括同型半胱氨酸升高)抗凝蛋白缺乏，尤其是AT缺乏抗磷脂抗体综合征获得性危险因素持续存在反复发作的VTED-