Hematology and Oncology Book

http://www.fpnotebook.com/

Protein C DeficiencyAka: Protein S Deficiency

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  1. 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
  2. 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

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