南方医科大学：《内科学》课程教学课件（讲稿）第六篇 血液系统疾病 第十四章 出血性疾病 Hemorrhagic disorder（打印版）

250 出血性疾病 Hemorrhagic disorder 珠江医院血液科邓兰2020.4

Key words ·Hemorrhage,hemorrhagic出血，& 出血的 Hemostasis,hemostatic 止血，止血的 ·Blood coagulation 凝血 Fibrinolysis,fibrinolytic 纤溶，纤溶的

Contents ·Summary ·Allergic purpura Immune thrombocytopenia Thrombotic thrombocytopenic purpura ·Hemophilia

Chapter 1 --Summary

Factors of hemostasis 1.Blood vessel 2.Platelet --When it is injured its endothellal cells will release · By VWF,platelets adhere to VWF to make platelets adhere collagen fiber to form thrombus and aggregate there of platelets release TF to start extrinsic pathway of coagulation By fibrinogen,platelets aggregate collagen fiber of blood vessel exposed will activate FXIl,and Then the platelets activated start intrinsic pathway of excrete substance to regulate coagulation blood coagulation 血小板 GPI。-IX 纤维 WF 胶原 GPII。-a 蛋白原 血小板聚集

Factors of hemostasis 3.System of blood coagulation Process of coagulation 。 Blood clotting factors 组织损伤 接触激活 Extrinsic and intrinsic FXIa pathway of coagulation HMWK IXa PK Form thrombus of fibrin VaX XⅪ Xa 凝血酶原 纤维蛋白原⊙-0-0 纤维蛋00-可 凝血 交联的纤维蛋白 ↓x 00000可

Process of hemostasis 血管损伤 胶原暴露 组织因子释放 血管收缩 出血 血小板 粘附 凝血酶 血肿压迫血管 聚集 释放 血流减慢一血小板血栓 止血血栓 止血 纤溶酶 血管再通

System of anticoagulation Heparin-antithrombin(AT)system: AT which is the most important anticoagulant can inactivate FXa and Flla,and heparin can change the configuration of AT to promote anticoagulation Protein C system:including PC,PS and TM Inhibition of TF pathway Antithrombin Thrombin

System of fibrinolysis Plasminogen(PLG):It can be activated into plasmin e t-PA and u-PA:they activate PLG into plasmin Inhibitions of plasmin 。 Fibrinolytic system can be activated through intrinsic or extrinsic pathway Fibrinogen and fibrin can be decomposed into fibrinogen degradation products(FDP),D- dimer,and so on

Contact Factor Pathway Tissue Factor Pathway Protein Concentrations (Intrinsic Pathway) (Extrinsic Pathway) Component o3盟r 品 Conce Kallikrein Prekallikrein "Tissue Damage" 330.000 120m 109 72.000 100 39 330.000 25 HK HK actor VIl 5000 Tissue Factor 160.000 Xila Factor Xll 80.000 中市市市 0375 (Vlla) TFPI Xa Factor Xill 320.000 0.0312 Ca++ 62,000 C-linhibito +a VI 5814 H 110.000 Ca++ (VIla) Fibronectin 450.000 Antithrombin lll 56000 IXa 6600 63.00 ☒c Proten C inhibtor 57.000 Alpha.-Macroglobulin 725.000 L Xa Fibrinolysis Protein Antithrombin Ca+ Plasminogen PL APC a ←Heparin Cofactor Il UK.SK a Throm PAI-1 Fibrinogen Fibrin Monomer Protein C Inhibitor Fibrin Polymer Plasmin Protein C →Xa ca→ a-2 Antiplasmin Fibrin Clot 412 South Lafayette South Bend,Indiana 46601 DIAGNOS Phone:(219)288-4377·Fa(219)288-2272 888-727-2729 FXDP FDP ·Thrombophi gene mutation ND-制ot0 ermined