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Coagulation Disorders

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Coagulation Disorders Presentation Transcript

  • 1. COAGULATION DISORDERS
    by: Noel C. Santos, M.D.
  • 2. Hemostasis
    Physiologic mechanisms to protect from blood loss.
    Stops bleeding from an injured site
    Maintains blood in the fluid state within the vascular compartment
    HEMORRHAGE
    THROMBOSIS
  • 3. Normal Hemostatic Mechanisms
    VASCULAR SYSTEM
    PLATELETS
    COAGULATION SYSTEM
    Intrinsic Pathway
    VIII, vWF, IX, XI, XII, prekallikrein, HMWK
    Extrinsic Pathway
    III, VII
    Common Pathway
    FIBRINOLYTIC SYSTEM
  • 4. Primary Phase – formation of platelet plugs
    Secondary Phase – formation of fibrin plugs
  • 5. Bleeding Disorders
    SPONTANEOUS - petechiae, purpura, mucous membranes, GI bleeding, hematuria, into joint spaces
    EXCESSIVE - after trauma or surgery
    - range is from lethal diseases (factor VIII deficiency, Bernard-Soulier's, Glanzmann's) to asymptomatic (factor XII deficiency, many von Willebrand's)
  • 6. INCREASED VASCULAR FRAGILITY
    seldom serious
    skin bruises, dependent petechiae, gum bleeding, hematuria, nosebleeds, GI bleeds.
    Infections - damage endothelium
    Amyloidosis
    Collagen problems
    Immune-complex deposition in vessel walls
  • 7. PLATELETS DEFICIENCY or DYSFUNCTION
    apparent immediately after trauma (petechiae – skin & mucus membranes)
    Thrombocytopenia
    count is less than 100 x 109/liter
    bleeding after trauma = platelet count is below 40 x 109/liter
    spontaneous bleeding = count is below 20 x 109/liter (petechiae &purpura randomly over the skin, blood blisters in the mouth, GI, GU, CNS bleeding.)
    severe spontaneous bleeding = when count gets < 10 x 109/liter
  • 8.
  • 9.
  • 10.
  • 11.
  • 12.
  • 13. Thrombocytopenia
    Causes:
    Decreased production
    Decreased survival
    Sequestration
    Dilutional
    Increased utilization
  • 14.
  • 15.
  • 16.
  • 17.
  • 18.
  • 19.
  • 20. Defective platelet functions
    Congenital
    Adhesion (Bernard-Soulier syndrome)
    Aggregation (thrombasthenia - Glanzmann’s)
    Secretion
    Acquired
    Aspirin ingestion
    Uremia
  • 21. DERANGEMENTS OF CLOTTING FACTORS
    apparent only few minutes after trauma
    deficiency can be mild or severe
    absent or defective or the deficiency may be due to an inhibitor against it
    hereditary (single) or acquired (several)
    ecchymoses or hematomas after minor injury, bleeding for days after tooth extractions, or bleeding into joint spaces (hemarthroses)
  • 22.
  • 23. Hemophilia
    Hemophilia A – deficient Factor VIII
    Sex-linked
    levels less than 1% of normal
    Hemophilia B – deficient Factor IX (Christmas disease)
    Sex-linked
    Assay of corresponding factor/s
  • 24. Von Willebrand’s Disease
    Autosomal dominant
    Type I – most common, inhibited release of multimers by unknown mechanisms, less amount is available
    Type II – defective multimers are released
    Type III – none is produced
    Usually associated with reduced Factor VIII
  • 25. FIBRINOLYTIC Problems
    Excessive plasminogen activation
    Elevated fibrin degradation products
    DISSEMINATED INTRAVASCULAR COAGULATION
    Thrombohemorrhagic disorder
    SECONDARY COMPLICATION
    activation of coagulation sequence
    microthrombi formation with consumption of platelets, fibrin & coagulation factors
    activation of fibrinolytic mechanisms
  • 26. DISSEMINATED INTRAVASCULAR COAGULATION
    signs and symptoms relating to infarction
    hemorrhagic diathesis due to depletion of platelets and clotting factors AND activation of fibrinolytic mechanisms
    Mechanisms:
    release of tissue factor and thromboplastic substances into the circulation
    endothelial injury
  • 27. HYPERCOAGULABLE BLOOD – Thrombosis
    "Thrombophilia“, "hypercoagulopathy“
    Not rare, but tends to get overlooked.
    deficient natural anticoagulants (Protein C, Protein S and Antithrombin III)
    Sticky platelet syndrome
    Dysfibrinogenemia - mutant fibrin is not removed by plasmin
    plasminogen deficiency
  • 28.
  • 29. Laboratory Screening Tests
    Primary Phase – Bleeding Time
    Secondary Phase – Clotting Time
  • 30. Laboratory Screening Tests
    VASCULAR SYSTEM – capillary fragility testing (tourniquet test)
    PLATELETS – count and function (CRT)
    COAGULATION SYSTEM
    Intrinsic Pathway – APTT
    Extrinsic Pathway – Prothrombin time
    Common Pathway – Thrombin time
    FIBRINOLYTIC SYSTEM – FDP, antithrombin, natural anticoagulants
  • 31. Laboratory Tests – SPECIFIC
    Platelet Function Tests
    Assays of Clotting Factors and Anticoagulants
    Detections of antibodies against coagulation elements
  • 32. SUMMARY
    Tourniquet Tests – (+) or (-)
    Bleeding Time – (N) or (P)
    Clotting Time – (N) or (P)
    Platelet Count – (N) or Dec or Inc
    Clot Retraction Time – (N) or Abn
    Prothrombin Time – (N) or (P)
    Activated Partial Thromboplastin Time – (N) or (P)
    Thrombin Time – (N) or (P)
    What Specific Tests?
  • 33. COAGULATION DISORDERS
    by: Noel C. Santos, M.D.