Protein C Deficiency

Aka: Protein C Deficiency, Protein S Deficiency

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II. Pathophysiology

  1. Protein C and Protein S common features
    1. Both are Vitamin K dependent
    2. Glycoproteins synthesized by the liver
    3. Endogenous Anticoagulants
  2. Protein C neutralizes factors V and VIII (ultimately preventing Thrombin formation)
    1. Deficiency inherited Autosomal Dominant
    2. Venous thrombosis occurs if functional levels <50%
    3. Associated with venous thrombosis in 1 in 20,000
  3. Protein S is Cofactor for Protein C
    1. Venous thrombosis occurs if functional levels <60%

III. 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

IV. References

  1. Jean-Louis and Sethuraman (2023) Crit Dec Emerg Med 37(7): 4-11

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