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Hemostasis :. Hemo = خون . Stasis = سکون. Hemostasis : Hemo/Stasis. مثلث هموستاز:. Platelets. Blood Vessels. Hemostatic Factors. Primary: ITP Neonatal Isoimmune TAR Syndrome Wiskott-Aldrich Syn. Secondary: *Malignancy *Aplastic Anemia *DIC *Sepsis *HUS *Hypersplenism
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Hemo=خون Stasis=سکون Hemostasis:Hemo/Stasis
مثلث هموستاز: Platelets Blood Vessels Hemostatic Factors
Primary: ITP Neonatal Isoimmune TAR Syndrome Wiskott-Aldrich Syn. Secondary: *Malignancy *Aplastic Anemia *DIC *Sepsis *HUS *Hypersplenism *Autoimmune(SLE) Causes of Bleeding(1)Thrombocytopenia:
Primary: vWF Deficiency Hemophilia Platelet dysfunction Secondary: DIC Anticoagulants Vit K deficiency Hepatic Failure Renal Failure Maternal Anticonvulsant Causes of Bleeding(2)Coagulopathy:
Causes of Bleeding(3)Vascular(Non-Hematologic) • Child Abuse • Vasculitis • Ulcer • Varices • Ehlers-Danlos Syndrome • Telangiectasia • Angiodysplasia
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.
Estimation of BT with desired Platelet count BT= 30.5- Platelet count (minute) 3,850
Vitamin K Related Factors: • Factor II • Factor VII • Factor IX • Factor X
Prolonged PTT • No clinical bleeding ??? • Mild or rare bleeding ??? • Frequent,Severe Bleeding ???
Prolonged PTT • No clinical bleeding Factor XII , HMWK , PK • Mild or rare bleeding Factor XI • Frequent,Severe Bleeding Factors VIII and IX
Prolonged PT • ??? • ??? • ???
Prolonged PT • Factor VII Deficiency • Vitamin K Deficiency(Early) • Warfarin anticoagulant ingestion
Prolonged PT and PTT • ??? • ??? • ???
Prolonged PT and PTT • Factor II,V,X Deficiency • Vitamin K Deficiency(Late) • Warfarin anticoagulant ingestion
Prolonged TT • ??? • ??? • ???
Prolonged TT • Mild or rare bleeding: Afibrinogenemia • Frequent,Severe Bleeding:Dysfibrinogenemia • Heparin like inhibitors or heparin administration
Prolonged PT and/or PTT not corrected with normal plasma • Specific or nonspecific inhibitor Syndromes
Clot Solubility in 5 M urea • Factor XIII deficiency • Inhibitor
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?
Clinical approach 1. Is the bleeding significant ? 2. Local Vs Systemic ? 3. Platelet Vs Coagulation disorder ? 4. Inherited Vs Acquired ?
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
Screening Tests 1. Platelet count & morphology 2. Bleeding Time(BT) 3. Prothrombin Time(PT) 4. Activated Partial Thromboplastin Time(PTT) 5. Thrombin Time (TT)
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