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  1. Hemostasis:

  2. Hemo=خون Stasis=سکون Hemostasis:Hemo/Stasis

  3. مثلث هموستاز: Platelets Blood Vessels Hemostatic Factors

  4. Primary: ITP Neonatal Isoimmune TAR Syndrome Wiskott-Aldrich Syn. Secondary: *Malignancy *Aplastic Anemia *DIC *Sepsis *HUS *Hypersplenism *Autoimmune(SLE) Causes of Bleeding(1)Thrombocytopenia:

  5. Primary: vWF Deficiency Hemophilia Platelet dysfunction Secondary: DIC Anticoagulants Vit K deficiency Hepatic Failure Renal Failure Maternal Anticonvulsant Causes of Bleeding(2)Coagulopathy:

  6. Causes of Bleeding(3)Vascular(Non-Hematologic) • Child Abuse • Vasculitis • Ulcer • Varices • Ehlers-Danlos Syndrome • Telangiectasia • Angiodysplasia

  7. تقسیم بندی هموستاز: 1)هموستاز اولیه:چند ثانیه بعد از آسیب عروقی ایجاد میشود و از خونریزی از عروق کوچک و ونولهاجلوگیری میکند. 2)هموستاز ثانویه:چند دقیقه بعد از آسیب عروقی ایجادمیشود و از خونریزی از عروق بزرگ جلوگیری میکند.

  8. Differences of Primary and Secondary Hemostasis:

  9. Estimation of BT with desired Platelet count BT= 30.5- Platelet count (minute) 3,850

  10. Vitamin K Related Factors: • Factor II • Factor VII • Factor IX • Factor X

  11. Prolonged PTT • No clinical bleeding ??? • Mild or rare bleeding ??? • Frequent,Severe Bleeding ???

  12. Prolonged PTT • No clinical bleeding Factor XII , HMWK , PK • Mild or rare bleeding Factor XI • Frequent,Severe Bleeding Factors VIII and IX

  13. Prolonged PT • ??? • ??? • ???

  14. Prolonged PT • Factor VII Deficiency • Vitamin K Deficiency(Early) • Warfarin anticoagulant ingestion

  15. Prolonged PT and PTT • ??? • ??? • ???

  16. Prolonged PT and PTT • Factor II,V,X Deficiency • Vitamin K Deficiency(Late) • Warfarin anticoagulant ingestion

  17. Prolonged TT • ??? • ??? • ???

  18. Prolonged TT • Mild or rare bleeding: Afibrinogenemia • Frequent,Severe Bleeding:Dysfibrinogenemia • Heparin like inhibitors or heparin administration

  19. Prolonged PT and/or PTT not corrected with normal plasma • Specific or nonspecific inhibitor Syndromes

  20. Clot Solubility in 5 M urea • Factor XIII deficiency • Inhibitor

  21. Secondary Hemostasis Approach: 1)What is diagnosis? 2)What is hemostatic level of Factor? 3)What is blood distribution of factor? 4)Which products contain desired factor? 5)What is half life of coagulation factor?

  22. APPROACH TO COAGULATION DISORDERS

  23. Clinical approach 1. Is the bleeding significant ? 2. Local Vs Systemic ? 3. Platelet Vs Coagulation disorder ? 4. Inherited Vs Acquired ?

  24. Laboratory Approach 1. Demonstration of the defect 2. Identification of the defect(s) 3. Assessment of severity 4. Consequential studies eg. carrier detection 5. Monitoring of treatment

  25. Screening Tests 1. Platelet count & morphology 2. Bleeding Time(BT) 3. Prothrombin Time(PT) 4. Activated Partial Thromboplastin Time(PTT) 5. Thrombin Time (TT)

  26. Collection of blood sample 1. Minimum circulatory stasis 2. Clean venous puncture 3. Proper anticoagulant 4. Proportion of blood to anticoagulant 5. Separation of plasma and storage 6. Effect of stress, pregnancy, drugs 7. Effect of PCV on the proportion of plasma to anticoagulant