复旦大学附属中山医院：出血性疾病 DISORDERS OF HEMOSTASIS

DISORDERS OF HEMOSTASIS Dept of Hematology ZhongShan Hospital, FuDan University Prof, JIAn-mInXU

DISORDERS OF HEMOSTASIS Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU

General Considerations Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding menorrhagia, gastrointestinal bleeding purpura, and petechiae

General Considerations • Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation) • Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding, menorrhagia, gastrointestinal bleeding, purpura, and petechiae

General Considerations Petechiae are seen almost exclusively in conditions of thrombocytopenia and not platelet dysfunction Bleeding due to coagulopathy may occur as deep muscle hematomas as well as skin bleeding Spontaneous hemarthroses are seen only in severe hemophilia

General Considerations • Petechiae are seen almost exclusively in conditions of thrombocytopenia and not platelet dysfunction. • Bleeding due to coagulopathy may occur as deep muscle hematomas as well as skin bleeding. • Spontaneous hemarthroses are seen only in severe hemophilia

General Considerations Definition: disorder of hemostasis spontaneous bleeding abnormal bleeding following trauma · Machanism: vessel wall defect thrombocytopenia, platelet disfunction coagulation disorder

General Considerations • Definition: disorder of hemostasis spontaneous bleeding abnormal bleeding following trauma • Machanism ： vessel wall defect thrombocytopenia, platelet disfunction coagulation disorder

Normal hemostasis and coagulation hemostasis: 1. Vessel wal (1)vessel spasm: reflex spasm SHT↑ slow down blood flow (2) subendothelial connective tissue encouraging platelet adhesion, activation aggregation (3) releasing TF, activating FXII triggering intrinsic and extrinsic pathway (4) pression of haematoma

Normal hemostasis and coagulation • hemostasis: 1. Vessel wall： （1）vessel spasm: reflex spasm S-HT↑ slow down blood flow （2）subendothelial connective tissue： encouraging platelet adhesion, activation, aggregation （3）releasing TF, activating FⅫ： triggering intrinsic and extrinsic pathway （4）pression of haematoma

Normal hemostasis and coagulation 2. platelet (1) adhesion to subendothelial connective tissue by GPi b、WF (2) aggregation formation of platelet thrombus by gPllb/Ⅲa (3 release phosphatide-Arachidonic Acid -TXA2 further platelet activation and adhesion violent vessel spasm

Normal hemostasis and coagulation 2. platelet： （1）adhesion： to subendothelial connective tissue by GPⅠb、vWF （2）aggregation ： formation of platelet thrombus by GPⅡb/Ⅲa （3）release： phosphatide-Arachidonic Acid -TXA2 further platelet activation and adhesion violent vessel spasm

Normal hemostasis and coagulation 3. coagulation: (1)Rearrangement of platelet membrane phosphatidate-PF3 (2) Tissue injury- TF release (3) Subendothelial| connective tissueⅫ-Ⅻa triggering intrinsic and extrinsic pathway- white thrombus-red thrombus

Normal hemostasis and coagulation 3. coagulation: （1） Rearrangement of platelet membrane phosphatidate-PF3 （2） Tissue injury- TF release （3） Subendothelial connective tissue-Ⅻ-Ⅻa triggering intrinsic and extrinsic pathway￾white thrombus-red thrombus

Normal hemostasis and coagulation coagulation 1. nature: a cascade of enzymes activation not active before activated 2. component 1.12 coagulation factor protein( exclude ca×O 11 in plasma( tissue factor) code with IV etc 2. PK R 3. HMWK

Normal hemostasis and coagulation • coagulation： 1. nature: a cascade of enzymes activation not active before activated 2. component: 1. 12 coagulation factor: 11 in plasma (tissue factor) protein ( exclude Ca++ ) code with Ⅰ、Ⅱ、Ⅲ、Ⅳ etc. 2. PK 3. HMWK

Normal hemostasis and coagulation 3. course (1) thromboplastin intrinsic: XI- XIXIa-iX Ia+Ⅷ山+ca+PF3 extrinsic: ll-Vll+Ca X-Xa+V+Ca+PF3 (2) thrombin: prothrombinthromboplatin+Cathrombin

Normal hemostasis and coagulation 3. course： （1）thromboplatin intrinsic: Ⅻ-Ⅻa——Ⅺ-Ⅺa-Ⅸ- Ⅸa+Ⅷ+Ca+PF3 extrinsic: III-VII+Ca Ⅹ-Ⅹa+Ⅴ+Ca+PF3 （2）thrombin：prothrombinthromboplatin+Cathrombin

Xa因子的核心作用 内源性系统 外源性系统 细胞的促凝血 XIa 激 Vila X IXa Ca2+ Ca PL Xa X Xa Va Ca2+ 凝血酶 PL lla 纤维蛋白原 溶解性纤维蛋白纤维蛋白(凝块) Boneuet al. Sang Thrombose Vaisseaux. 1998; 10. 291-313

Boneu et al. Sang Thrombose Vaisseaux. 1998;10:291-313. II VIIa 细胞的促凝血酶 原激酶 Ca2+ Xa XIa 内源性系统 外源性系统 X IX XIIIa XI XII XIIa 纤维蛋白原 溶解性纤维蛋白 纤维蛋白（凝块） Xa Va PL Ca2+ VII 凝血酶 IIa IXa VIIIa Ca2+ PL Xa因子的核心作用