Treatment of Protein C Deficiency with Disseminated Intravascular Coagulation, Purpura Fulminans, or Acute Venous Thromboembolism

Clinical Scenario

Severe protein C deficiency (protein C activity <1 IU/dL) is a rare autosomal recessive disorder that typically presents with purpura fulminans and severe disseminated intravascular coagulation, often accompanied by venous thromboembolism. This combination of findings defines an acute, high-risk clinical presentation requiring prompt, structured intervention.

Treatment Approach

Acute management centres on protein C replacement therapy given alongside therapeutic anticoagulation. Where protein C replacement is not immediately available, an interim plasma-based approach can provide a bridge until definitive replacement becomes accessible.

The complete regimen — including replacement strategy, anticoagulation agent selection, sequencing, and the full monitoring protocol — is detailed in the structured evidence-based protocol below.

Clinical Targets

Monitoring is directed at confirming adequate protein C activity levels, tracking D-dimer trends as a marker of ongoing coagulation activation, and verifying that therapeutic anticoagulation targets are reached. Sustained improvement across these parameters guides titration and ongoing management.