复旦大学：《内科学 Internal Medicine MBBS》课程教学资源（课件讲稿）Hemotologic System_Disorders of coagulation and thrombosis introduction and Hemephilia

DISORDERS OF COAGULATION AND THROMBOSIS Yunfeng Cheng D Zhongshan Hospital, Fudan University

DISORDERS OF COAGULATION AND THROMBOSIS Zhongshan Hospital,Fudan University Yunfeng Cheng

Major Components in Coagulation Vessel wall 2 Platelet 3/ Coagulation factor cascade Clot inhibition/lysis

Major Components in Coagulation 1 Vessel Wall 2 Platelet 3 Coagulation factor cascade 4 Clot inhibition/lysis

PRIMARY Vascular damage HEMOSTASIS L/Tissue destruction/+VWE+ Platelets Platelet plu pl Contact factor activation formation (Bradykinin HMWK) 业、EXa Tissue factor (TF) FIla TF/FVIla FXI FXIa Phospholipid FIX FVa EVIl FX Ca* F Xa sECoNDAR othrombin-Thrombin Fibrinolytic pathway Fibrinogen - Fibrin monomer Plasminogen HEⅣo PAl-1it-PAUrokinase PA|-1 Plasmin Cross-linked Fibrin polymer D-dimer The coagulation cascade Fibrin degradation Asls products(FDPs

coagulation cascade The coagulation cascade

XIa The coagulation cascade Activated a + VIlla proteinCxProtein Thrombomodulin Tf-VII Thrombin Fibrinolysis Plasmin 、 Tissue factor. Antithrombin Thrombin… athway inhibitor activatable fibrinolysis

The coagulation cascade

The coagulation cascade The coagulation cascade of secondary hemostasis has two pathways which lead to fibrin formation contact activation pathway (the intrinsic pathway) tissue factor pathway(the extrinsic pathway It was previously thought that the coagulation cascade consisted of two pathways of equal importance joined to a common pathway It is now known that the primary pathway for the initiation of blood coagulation is the tissue factor pathway

The coagulation cascade The coagulation cascade of secondary hemostasis has two pathways which lead to fibrin formation. contact activation pathway (the intrinsic pathway) tissue factor pathway (the extrinsic pathway) It was previously thought that the coagulation cascade consisted of two pathways of equal importance joined to a common pathway. It is now known that the primary pathway for the initiation of blood coagulation is the tissue factor pathway

Hemostasis ●BT,(CT) PT BV Iniury . PTt Neural Damage/contact Contact Blood vessel Coagulation Constriction Aggregation Cascade Primary hemostatic plug Reduced Platelet Activation FIbrin Blood flow formation Platelet study Stable Hemostatic Plug Antibody tests Factor Assay I

Hemostasis BV Injury Platelet Aggregation Platelet Activation Blood Vessel Constriction Coagulation Cascade Stable Hemostatic Plug Fibrin formation Reduced Blood flow Damage/contact. Primary hemostatic plug Neural •CBC-Plt •BT,(CT) •PT •PTT Platelet study Antibody tests Factor Assay Contact

HEMOSTASIS Primary Hemostasis Blood vessel contraction Platelet Plug formation Secondary Hemostasis Activation of Clotting Cascade Deposition Stabilization of Fibrin Tertiary Hemostasis Dissolution of fibrin clot Dependent on Plasminogen Activation

HEMOSTASIS Primary Hemostasis – Blood vessel contraction – Platelet Plug Formation Secondary Hemostasis Activation of Clotting Cascade Deposition & Stabilization of Fibrin Tertiary Hemostasis Dissolution of Fibrin Clot Dependent on Plasminogen Activation

Clinical Approach to Bleeding Disorders Clinical Evaluation(who, when, where, what) Laboratory studies Approach to The Patient

Clinical Approach to Bleeding Disorders Clinical Evaluation(who, when, where, what) 2 Laboratory Studies Approach to The Patient

Tests of hemostasis "Screening tests Bleeding. T-10m. Platelet BV function Prothrombin,- Extrinsic, aPtt- Intrinsic Thrombin T-common path. DIC ° Specific tests: Factor assays - hemophilia Tests of thrombosis- Tt FDp Platelet function studies. Adhesion, Aggregation, Release tests Bone marrow study

Tests of Hemostasis: • Screening tests: – Bleeding.T - 10m. Platelet & BV function – Prothrombin.T – Extrinsic, APTT – Intrinsic – Thrombin.T – common path. (DIC) • Specific tests: – Factor assays – hemophilia. – Tests of thrombosis – TT, FDP, – Platelet function studies: • Adhesion, Aggregation, Release tests. – Bone Marrow study

Bleeding: Clinical Features Local-Vs-General, spontaneous Hematoma/ Joint Bleeds-Coag Skin/Mucosal bleeds-PLT wound/surgical bleeding Immediate-PLT Delayed -Coagulation

Bleeding: Clinical Features • Local - Vs - General, spontaneous . . • Hematoma / Joint Bleeds- Coag • Skin / Mucosal Bleeds – PLT • wound / surgical bleeding – – Immediate - PLT – Delayed - Coagulation