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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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