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Haemostasis, ITP, Thrombotic microangiopathies, Leading to - Coggle Diagram

- - - - Stop codon in exon 5
      - Levels ~1%
    - - Phe283 replaced with Leu (missense mutation)
      - Levels ~10%
- - - - Joints
      - Muscles
      - Brain
    - - Increased pressure
  - - - Beriate
      - Increased T\(\frac{1}{2}\)
        
        Elocta
        
        Novo8
    - - Development of inhibitors (10-15%)
    - - Novel Ab that mimics FVIII
      - Subcutaneous injection for severe haem A
      - Monitored by modified FVIII assay
- - - - Immunosuppressants (prednisone, cyclophosphamide)
      - Novoseven for bleeds
- - - - Monomers polymerise
  - - - On platelet surface
      - Activates FIX to enhance FXa generation
    - - Platelets change shape
      - Stabilisation of platelet plug
    - - Bind to fibrin + protected from inhibition by antithrombin
- - - - FFP
      - Octaplas
- - - - Inhibit fibrin polymerisation + platelet aggregation
- - - - Thrombin T\(\frac{1}{2}\)=<1 minute
  - - - No longer able to cleave fibrinogen
    - - Rapidly dissociates so thrombomodulin can bind thrombin + activate protein C
        
        Activated protein C + S inactivate Va + VIIIa
        
        Confines thrombin generation to site of injury
- - - - Thrombocytopenia
        
        Normal platelet count 150-350x10\(^9\)/L
        
        <150x10\(^9\)
        
        Mild (50-150), moderate (20-50) or severe (<20)
        
        Bleeding seldom occurs >50x10\(^9\)/L
        
        Minor >10x10\(^9\)/L
        
        Spontaneous <10x10\(^9\)/L
        
        Clinical bleeding doesn't always correlate to platelet count
        
        Other factors e.g. endothelium integrity + platelet functionality
        
        Diagnosis
        
        Full blood count
        
        Blood film
        
        Platelet clumping - citrate sample
        
        Large platelets - congenital thrombocytopenias e.g. BS or MH or ^ turnover e.g. ITP or consumption syndrome
        
        Small platelets - Wiscott-Aldrich
        
        Red cell fragments - thrombotic microangiopathy
        
        Hypogranular neutrophils - myelodysplasia
        
        Etiology
        
        Exclude congenital thrombocytopenia's - previous normal count
        
        Exposure to drugs e.g. alcohol or quinine
        
        Viral infections e.g. HIV/CMV
        
        Post-operative - dilutional - will resolve, cardiac - may persist
        
        Autoimmune e.g. ITP, anti-phospholipid syndrome + post transfusion purpura (alloantibodies)
      - Thrombocytosis
    - - Functional defect can be inherited or acquired
        
        Bernard Soulier GP 1b-IX-V
        
        Thrombocytopenia
        
        Large platelets
        
        Impaired binding of VWF
        
        Poor platelet adhesion + aggregation
        
        Glanzmann thrombasthenia GP IIb/IIIa
        
        Fails to bind fibrinogen
        
        Platelet count is normal
        
        No aggregation with agonists e.g. ADP, epinephrine + collagen
    - - Drugs
      - Alcohol
      - Uremia
      - Myeloproliferative disorders