Coags

Protein C Deficiency

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Protein C Deficiency, Protein S Deficiency

  • Pathophysiology
  1. Protein C neutralizes factors V and VIII
    1. Deficiency inherited Autosomal Dominant
    2. Venous thrombosis occurs if functional levels <50%
  2. Protein S is Cofactor for Protein C
    1. Venous thrombosis occurs if functional levels <60%
  3. Protein C and Protein S are both Vitamin K dependent
  • Complications
  1. Warfarin induced skin necrosis
    1. Protein C (short half life)
      1. Microvascular thrombi to skin
    2. Administer Heparin before Warfarin
      1. Eliminates risk of this complication
  2. Purpura fulminans
    1. Infants Homozygous for Protein C Deficiency