易栓症——神内病例.ppt
易栓症,Normal hemostasis（止血）,adhesion 粘附,aggregation 聚集,coagulation pathway 凝血途径,Coagulation balance,anticoagulantmechanisms,procoagulantmechanisms,fibrin formation,Fibrinmonomer,Endothelial cell surface,coagulation factors anticoagulant proteinsgain-of-function mutations fibrinolysis antiphospholipid Ab,fibrin formation,anticoagulantmechanisms,procoagulantmechanisms,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,Annual incidence of DVT,Whites 0.8-1.2 x 1000Hong Kong Chinese 0.16 x 1000 Liu et al,Hong Kong Med J 2002,rate ratio(vs white)African Americans 1.27(1.07-1.51)Hispanic 0.60(0.54-0.67)Asians/Pacific Islanders 0.26(0.22-0.30)*White et al,Ann Intern Med 1998,*idiopathic or secondary,VTE:a multifactorial disease,acquired,genetic,mixed,transient,VTE,risk factors,the old story.,deficiency of anticoagulant proteins year of discovery antithrombin 1965 protein C 1981 protein S 1984,ANTITHROMBIN,PROTEIN C,PROTEIN S:type of deficiency,Type I:Quantitative deficiency,Type II:Qualitative deficiency,antigenic test,functional test,functional test,=antigenic test,Prevalence of deficiencies,general unselected population VTE patients antithrombin 0.02-0.2%1%protein C 0.1-0.5%3%protein S?1-2%,Risk of VTE associated with deficiencies,increase relative risk antithrombin 5-50 protein C 7-15 protein S 6-10,year of discovery resistance to activated protein C 1993/94and factor V Leiden(G1691A)prothrombin mutation(G20210A)1996,the old story.,older agecancerantiphospholipid antibodiesprevious venous thromboembolism,Age and VTE,0-9,10-19,20-29,30-39,40-49,50-59,60-69,70-79,79,600,500,400,300,200,100,0,Anderson et al,1991,Incidence Rate per 100,000,Females,Males,Cancer and VTE,Tissue factor,constitutive of malignant cells,promotes angiogenesis and increases plasmin production(metastases)Prothrombotic cancer substances(cancer procoagulant,IL1,TNF,etc.)The risk of VTE is higher if chemotherapy(tamoxifen,thalidomide,L-asparaginase)VTE and occult cancer,year of discoveryhyperhomocysteinemia 1994high factor VIII 1995,the old story.,surgery and major traumaprolonged immobilizationpregnancy/puerperium(6 weeks postpartum)oral contraceptives/hormone replacement therapy,year of discoveryActivated protein C resistance 1993Air travel 1999,F：family history（尤其是强家族史，即家族中有至少两例同类型血栓患者）U：unusual locations of thrombosis（腹腔、颅内等部位）R：recurrent episodes of thrombosisY：year（45y）符合上述任1条均建议筛查易栓症指标,FURY,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 F VIII:C 蛋白C活性 蛋白S活性 抗凝血酶活性 APC-R F V Leiden 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,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。,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。,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VTE D-二聚体居高不下少见部位VTE较强血栓形成家族史,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VTE D-二聚体居高不下 少见部位VTE较强血栓形成家族史,建议该患者终生抗凝！,谢 谢,
