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Updated: Dec 21 2021

Protein C/S Deficiency

Images
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  • Snapshot
    • A newborn baby girl with no birth complications develops red purpuric lesions on the buttocks, elbows, and heels. She is lethargic and does not respond to soothing ointments. A pediatric dermatologist is consulted. By the time, the specialist sees the newborn, black eschars have formed on the pressure points. She is diagnosed with purpura fulminans and dies shortly after.
  • Introduction
    • Hypercoagulable state/thrombophilia caused by deficiency in protein C or S
    • Mnemonics
      • skin or subcutaneous necrosis after administration of warfarin
        • remember, warfarin affects protein C and S first
      • protein C deficiency is more common than protein S deficiency
      • “protein C Cancels Coagulation”
  • ETIOLOGY
    • Pathogenesis
      • review of anticoagulation pathway
        • protein C (with cofactor protein S) inactivates factors V and VIII
        • remember, both are vitamin K-dependent
        • both are synthesized in the liver
      • deficiency of either protein C or S → active factors V and VIII
        • thrombosis
    • Genetics
      • inherited
        • protein C deficiency - autosomal dominant
        • protein S deficiency – autosomal dominant (much less common)
      • acquired
        • decreased synthesis
          • vitamin K antagonist administration
          • severe liver disease with synthetic dysfunction
  • Presentation
    • Symptoms of thrombosis
      • after warfarin initiation
        • skin and subcutaneous necrosis
      • DVT or PE at a young age
      • increased risk of DIC
    • In severe cases
      • purpura fulminans in newborns
        • red purpuric lesions at pressure points
        • progresses to painful black eschars
  • STUDIES
    • Protein C or S functional assay (preferred)
      • ↓ protein C or S activity levels
    • Plasma protein C or S antigen levels
      • ↓ protein C or S antigen levels
    • Genetic testing not routinely done
  • Differential Diagnosis
    • Factor V Leiden mutation
    • Antithrombin deficiency
    • Antiphospholipid syndrome
    • HIT
  • Treatment
    • Prevention
      • avoid warfarin
      • avoid conditions that increase risk of thrombosis
        • OCPs
    • For thrombosis
      • protein C concentrate
      • anticoagulate with heparin
    • For warfarin-induced necrosis
      • stop warfarin
      • administer IV vitamin K
      • administer heparin
      • administer protein C concentrate or fresh frozen plasma
  • Complications
    • Miscarriage
    • Thrombosis
    • neonatal purpura fulminans
  • Prognosis
    • Severe disease associated with neonatal mortality
    • Risk of warfarin-induced skin necrosis
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