七年制出血疾病课件.ppt

Hemorrhagic diseases Classification of hemorrhagic diseases 1. Abnormality of blood vessel 2. Abnormality of platelets 3 . Abnormality of coagulation Mechanism of cougulation Coagulation factors FI，fibrinogen FII，prothrombin FIII，tissue factor，tissue thromboplastin FIV，Ca++ FV, labile factor FVII, stable factor FVIII, antihemophilic globulin, AHG FIX, plasma thromboplastin component,PTC， Christmas factor FX, Stuart-Prowe factor FXI, plasma thromboplastin anticedent,PTA FXII, Hageman Factor FXIII, fibrin stablizing factor PK HMWK Coagulation cascade?theory The mechanism of anticoagulation and fibrinolysis The system of anticoagulation *Antithrombin (AT) *Protein C system *Tissue factor pathway inhibitor (TFPI) *Heparin Fibrinolysis system *Plasminogen (PLG) *t-PA *u-PA *Plasmin-related inhibitor ? Laboratory examination for hemorrhagic diseases 1. Platelet count 2. Bleeding time(BT) 3. Clot retraction test 4. Capillary fragility test ? If above items are abnormal ,that means abnormality of blood vessel or platelet . 5. Clotting time(CT) 6. Plasma prothrombin time (PT) 7. Thrombin clotting time(TT) 8. Activation partial thromboplastin time(APTT) Kaolin partial thromboplastin time(KPTT) ? If above items are abnormal, that means abnormality of coagulation. PT FVII deficiency APTT hemophilia or FXI deficiency PT deficiency of FV, FX, APTT FII, or fibrinogen abnormalities. Acquired deficiencies of plasma coagulation are more frequent than congenital disorder; the most common disorders include : --Hemorrhagic diathesis of liver disease; --Disseminated intravascular coagulation (DIC), --Vitamin K deficiency of more than one clotting factor. treatment Requires repla