SEVERE PROTEIN-C DEFICIENCY IN MENINGOCOC CAL SEPTICEMIA WITH PURPURAFULMINANS

In a five year old girl who developed menigococcal septicemia and pupu ra fulminans in the course of one day coagulation analysis showed cons umption coagulopathy with protein C deficiency at the time of admissio n. In addition to intensive supportive measures and antibiotic therapy , freshly frozen plasma, concentrates of factors II, VII, IX, and X co ntaining protein C, as well as antithrombin III were substituted. The disturbed plasmatic coagulation was corrected, but microcirculatory st asis was not influenced. Therefore, amputation of several limbs was ne cessary. Our case as well as other case reports in the literature demo nstrate the importance of protein C deficiency in meningococcal septic emia. Substitution of coagulation factors as such does not seem to hav e an effect on the collapse of the microcirculation. Combined therapy using a specific thrombolytic agent such as recombinant tissue plasmin ogen activator and activated protein C may present a method to avoid t he subsequent effects of endothelial damage.