Protein C replacement in severe meningococcemia: Rationale and clinical experience

Severe meningococcemia, which is associated with hemodynamic instability, p urpura fulminans and disseminated intravascular coagulation, still has a hi gh mortality rate, and patients who survive are often left invalids because of amputations and organ failure. Clinical studies have shown that levels of protein C are markedly decreased in patients with severe meningococcemia and that the extent of the decrease correlates with a negative clinical ou tcome. There is a growing body of data demonstrating that activated protein C, in addition to being an anticoagulant, is also a physiologically releva nt modulator of the inflammatory response. The dual function of protein C m ay be relevant to the treatment of individuals with severe meningococcal se psis. In the present review we give a basic overview of the protein C pathw ay and its anticoagulant activity, and we summarize experimental data showi ng that activated protein C replacement therapy clearly reduces the mortali ty rate for fulminant meningococcemia.