实验诊断学—止血凝血实验(双语版)

止血凝血2013年3月7日19:32血管壁止血作用：1.收缩反应：neurohumoral regulation→endothelial cells contract→slow blood flow→bleeding stops2.激活血小板Collagen exposed →activate vWF →collagen-vWF-platelet complex →platelet adheresion3.促进血液凝固过程Endothelial cells →tissue factor →extrinsic pathway of blood coagulationEndothelial cells →activate FXII →intrinsic pathway of blood coagulation4.抑制纤维蛋白溶解Endothelial cells →PAI(plasminogen activator inhibitor)一、血管壁检测——screening test1.BT2.CRT1.bleeding time, BT测定皮肤受特定条件的外伤后，出血自然停止所需的时间——反应毛细血管壁和血小板止血功能参考值：6.9±2.1minabnormal：>9minClinical significance:Influenced by the amount of platelet and the condition of the vascular wallBT increase:1.Abnormal function or amount of the platelet: thrombasthenia血小板无力症2.abnormal condition of the vascular wall: hereditary capillary rise syndrome3.intergrated factors: VWD、DICBT decrease:Severe hypercoagulable state and thrombotic diseases:Cerebral thrombosis、cardiac infarction、diabetes mellitus、hyperlipidemia、DVT、hypercoagulable state of DIC2.Capillary resistance test, CRT毛细血管的完整性与其本身的结构、功能，血小板的质、量，以及一些体液因素有关。

用加压的方法来部分阻止静脉血液回流，可以根据一定范围内新出血点的数目及大小来估计毛细血管的脆性。

Reference value: in the diameter of 5cmMale: less than 5 spotsFemale and Children: less than 10 spotsClinical significance:Positive when the amount of spots is more than the normal levelstructural or functional defect of the vascular wall:hereditary capillary rise syndrome、angiohemophiliaQuality and quantity defect of platelet:Thrombocytopenia 血小板减少症Platelet dysfunction syndrome 血小板功能缺陷症二、血小板检测1.keep the integrity of vascular wall and the permeability of capillary2.coagulationprimary hemostasis:Aggregation→adheresion→release→contractsecondary hemostasis:Provide the catalytic surface of the coagulation with PF3's participationScreening test:1.platelet countReference value: （100-300）×10^9/LClinical significance:Thrombopenia:Production dysfunction:Hematopoietic dysfunctionOver damage and consumption of platelet:ITP、DIC、TTPAbnormal distribution of platelet:Splenomegaly、dilution of bloodThrombocytosis:Primary increase: bone marrow incremental diseases( polycythemia vera真性红细胞增多症、primary thrombocythemia、early stage of Myelofibrosis、hemorrhage )Secondary increse: acute infection、hemolysis、certain kind of CA2.clot retraction test, CRT血液凝固后，由于血小板释放的PF4（血栓收缩蛋白），使血块收缩，血清析出。

测定析出血清量占全血量的百分数来表示血块收缩的程度，可间接了解血小板功能情况。

Reference value:定量法（凝块法）：37℃1小时其血块收率为65.8%±11.0%定性试验：37℃2小时开始收缩，18-24小时完全收缩（血清占全血1/3-1/2）。

Clinical significance:Clot retracting ability is based on the platelet、fribinogen,Fg、amount of RBCIncomplete retraction:→Quantity disorder of platelet:Thrombasthenia、ITP、thrombocytosis→disorder of fribinogen:Hypofribinogenosis→increase of RBC: erythrocytosisOver retraction:Congenital/acquired FXIII deficiency syndromeDiagnostic test3.platelet associated immunoglobulin, PAIg本试验是免疫性血小板减少性紫癜诊断、疗效及预后估计的重要指标，也有助于其他血小板疾病的免疫机制研究。

Reference value:PAIgG：（0～78.8）ng/10^7血小板PAIgM：（0～7.0）ng/10^7血小板PAIgA：（0～2.0）ng/10^7血小板Increase of PAIg:ITP、immune thrombocytopenic purpura4.platelet adhension test, PAdT血小板具有体内粘附于内膜下组织或体外粘附于带负电荷物质的性质。

当血液通过玻珠柱时，血小板可粘附在充满玻珠的塑料管内，血中血小板数会因此而降低。

Increase:Thrombotic diseaseDecrease:→Platelet factor: thromboasthenia→intergrated hemostasis factor: vWD→others: uremia、cirrhosis、MDS、AL、exotic protein increases5.platelet aggregation test, PAgT根据血小板聚集曲线的变化了解血小板聚集的程度和速度。

Increase: thrombotic diseasesDecrese:Congenital: thromboasthenia、vWDAcquired: myeloproliferative disorders、antiplatelet drugs三、凝血因子检测Screening test1.activated partial thromboplastin time, APTT( sensitive screening test of the intrinsive coagulation system)在受检血浆中加入活化的部分凝血活酶时间试剂和Ca2+，观察血浆凝固所需的时间Reference value:1、（31-43）秒2、与对照相差在10秒以内APTT increase:→congenital coagulation factor disorder1.intrinsic:VIII、IX、XI eg. Hemophilia2. common: X、V、II、FgI→acquirced coagulation factor disorder1.liver damage2.Vit K deficiency : obstructive jaundice3.hyperfribinolysis: DIC4.anti-coagulation drugs: warfarin heparinAPTT decreseThrombotic diseaseAPTT is the primary factor the monitor the use of heparin treatment2.clotting time,CT3.prothrombin time, PT在受检血浆中加入钙离子和组织因子（TF或组织凝血活酶），观察血浆的凝固时间即为凝血酶原时间Reference value:① PT秒数：直接测定的时间（12±1）s （超过对照3s 以上为异常）② PT比值（PTR) ：待测血浆PT/对照PTPTR ：1.0±0.05③国际正常化比值（INR）：INR＝PTRISI（ISI=国际敏感指数）INR：1.0±0.1PT increase:→congenital coagulation factor disorder1.extrinsic:VII2. common: X、V、II、FgI→acquirced coagulation factor disorder1.liver damage2.Vit K deficiency : obstructive jaundice3.hyperfribinolysis: DIC4.anti-coagulation drugs: warfarin heparinPT decreseThrombotic disease4.fribrinogen, Fg凝血酶比浊法：在受检血浆中加入一定量的凝血酶，使血浆中的纤维蛋白原转变为纤维蛋白，通过比浊原理计算Fg的含量。