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DISORDERS OF HEMOSTASIS

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)

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DISORDERS OF HEMOSTASIS

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

  2. 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

  3. 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.

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

  5. 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

  6. 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

  7. Normal hemostasis and coagulation • 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

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

  9. 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

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

  11. Normal hemostasis and coagulation （3）fibrin：fibrinogenthrombinfibrin monomer-polymerXIIIapolymer （4）function of thrombin： coagulation：activateⅠ、Ⅻ、 strengthen Ⅷ、Ⅴ platelet：aggregation, release fibrinolysis：activate plasminogen

  12. Normal hemostasis and coagulation • Anticoagulant system： 1. anticoagulin： （1）each coagulate factor and their complex has its own inhibitor （2）important： AT-Ⅲ：70% anticoagulant activity serine proteinase inhibitor Ⅱa. Ⅻa. Ⅺa. Ⅸa. DK

  13. Normal hemostasis and coagulation （3）protein C system： PCTMAPC+PS sterilizeVa. VIIIa. Inhibit PAI

  14. Physiology and Pathology APCI Ⅹa,trypsinize， TM+thrombin activator sterilize Ⅴa，Ⅷa PC APC release plasminogen Ca++ →fibrolysis Ca++ PSmodulate the activation of c’ acceleration inhibition APC：Activated Protein C

  15. Normal hemostasis and coagulation • fibrinolysis： • plasminogenplasminogen activator plasmin （PAI）（a2-AP） 2. fibrinolysis： fibrinogen→fragmentX →fragmentY →fragmentE ↘ ↘ ↘ （FDP）polypeptideABC fragmentD fragmentD

  16. Abnormality 1. Vessel wall： heredity：Telangiectasis aquired：deficiency of Vit C、 Vit P hypersensitivity：anaphylactoid purpura else：

  17. Abnormality 2. Platelet： （1）thrombocytopenia： production：AA, leukemia, infection, drug destruction ：ITP exhaust：TTP、HUS、DIC

  18. Abnormality （2）thrombocythemia ： primary：Primary hemorrhagic thrombocythemia secondary：CML, splenectomy , infection trauma （3）function deficiency： heredity ：thrombasthenia secondary：drug, renal failure liver disease

  19. Abnormality 3. Coagulation： （1）heredity：hemophilia、willabrand disease （2）aquired：liver disease、renal failure、 VitK deficiency、 DIC （3）anticoagulant agents：Ⅷ、Ⅸ-Ab、drug

  20. Diagnosis • History and physical exam(PE): 1.history：cause, sex、heredity background course 2.PE：petechial bleeding (organ, muscle, joint cavity)

  21. Diagnosis • Lab examination： 1.filter： vessel, platelet: BT、plt、CRT coagulation：CT、APTT、PT、TT

  22. Diagnosis 2.particular: （1）platelet： morphology：bulk、scatter、gathering adhesion、aggregation： inducer：Adr、ADP、collagen、ristocetin ↓ thrombasthenia ↑thrombus disease

  23. Diagnosis （2）Coagulation： APTT+CT：VIII. IX. XI PTCT：V. VII. X. II TT：fibrinogen↓、anticoagulant agents Coagulant factor test：quantity activation

  24. Prevention and Therapy 1. Prevention： drug：aspirin, persantin, indomethacin disease: liver disease ,renal failure heredity：prevent traumer 、operation

  25. Prevention and Therapy 2. hemostasis ： （1）supplyment：coagulant factor、fresh plasma、blood、platelet （2）drug： vessel：VitC、VitP、Carbazochrome 、Pred platelet：Etamsylate 、 Pred coagulation：VitK、prothrombin complex fibrinolysis：EACA,PAMBA,Tranexamic Acid （3）local：oppression、decrease movement

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