Platelet and coagulation disorders

Hemostasis
1. Sequence of events
  1. Fibrinolysis
    1. TPA
      1. Plasmin
        Fibrin
        FDP
        D-dimer
        Marker of PE
  2. Vasoconstriction
    1. Neurohumoral
    2. Endothelin
  3. Primary plug
    1. Platelet adhesion
      1. VWF binds to GIb on platelets
      2. VWF glues subendothelial collagen
      3. Platelet shape change
    2. Platelet activation
      1. Release of platelet contents
      2. Flip in the phospolipid membrane
      3. ADP activates the GIIb receptor
    3. Platelet aggregation
      1. Binding GIIb betn platelets with fibrinogen
  4. Secondary hemostatic plug
    1. Coagulation cascade on platelet membrane
      1. Instrinsic pathway
        Surface activation FXII
        PTT
        50: 5O Correction
        FVII, IX, XI, XII
        50:50 correction
        Circulating antibodies
        SLE, APLA
      2. Extrinsic pathway
        PT
        50:50 correction
        FVII
        50:50 no correction
        Antibodies
      3. Common pathway
        X-->Xa
        Proth--> Thrombin
        Fibrinogen--> Fibrin
    2. Clot stabilization
      1. FXIII
Thrombophilia
1. Virchows triad
  1. Hypercoagulability
    1. Factor V L mutation
      1. Va is resistant for inactivation by APC
    2. AT-III Def
      1. Thrombin uninhibited
    3. PT gene mutation
      1. Increased Prothrombin
    4. HIT
      1. Unfractioned heparin
      2. Thrombocytopenia
      3. Antibodies to PF-2
    5. APLA
      1. Antibodies to Glycoprotein I
      2. False + VDRL
      3. Rec PE
      4. Repeated abortions
      5. Libmann sack endocarditis
  2. Stasis
    1. Prolonged immobilization
  3. Endothelial dysfunction
    1. Smoking
    2. Atherosclerosis
Thrombocytopenia
1. Decreased production
  1. Marrow failure
  2. Leukemia or infiltration
2. Decreased survival
  1. Acute ITP
    1. Child after viral
      1. <20000 results in ICH
  2. Chronic ITP
    1. Antibodies to GPIb or GPIIb
    2. Seen in females,
    3. Diagnosis by exclusion
Quantitative disorder
Qualitative disorders
1. Bernard soulier
  1. Adhesion defect absent GPib
  2. RIPA reduced
  3. Platelet count normal
2. Glanzmann
  1. Platelet aggregation defect
  2. Defective GPIIb/IIIa
  3. Ristocetin -normal
VWD
1. VWF secreted from Endothelial cell
2. Adhesion defect
3. RIPA reduced
4. Desmopression increases release of VWF
MAHA
1. TTP
  1. Neurological
  2. Thrombocytopenia
  3. Renal failure
  4. Fever
  5. Hemolytic anemia
  6. Defect in ADMATS-13
2. HUS
  1. Endothelial dysfunction due to toxin
  2. After eating hamburger
  3. Renal failure
  4. No neurological manifestation
  5. ECOLI O157:H7
3. DIC
  1. Bleeding from iv canula
  2. Extensive endothelial injury
    1. TF release
  3. etiology
    1. Obstretic complications
    2. Sepsis
    3. severe burns
  4. Lab diagnosis
    1. Increased PT, PTT INR
    2. Low platelets
    3. Reduced fibrinogen
Coagulation factor defects
1. Hemophilia A
  1. Raised PTT
  2. 50:50 Mix correction
  3. FVIII DEF
  4. X- Recessive
2. Hmeophilia B
  1. FIX Deficiency
  2. XR

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Platelet and coagulation disorders

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	Created by Pooja Acharya over 4 years ago