Treatment of purpura fulminans in meningococcemia with protein C concentrate.
OBJECTIVE
To evaluate the clinical and laboratory effects of protein C concentrate as an adjunct to conventional therapy in the treatment of meningococcemia with purpura fulminans.


METHODS
Case series (pilot study).


METHODS
Intensive care unit in a tertiary care pediatric hospital.


METHODS
Four children (aged 3 months to 15 years) requiring intensive treatment for meningococcemia with shock, disseminated intravascular coagulation, and purpura fulminans.


METHODS
Intravenous administration of a protein C concentrate (100 IU/kg every 6 hours).


METHODS
Plasma protein C amidolytic activity, fibrinogen, and D-dimers; evolution of skin and limb lesions.


RESULTS
Treatment with protein C concentrate led to a rise in plasma protein C activity levels to within normal limits in all patients, associated with an increase in plasma fibrinogen and a bimodal decrease in D-dimers. No adverse effects were noted. All patients had reversal of organ dysfunction despite the severity of the initial illness. Two patients recovered completely with no sequelae; two required amputations.


CONCLUSIONS
These encouraging clinical and laboratory results and the absence of side effects warrant the initiation of a double-blind, randomized controlled multicenter trial to determine the role of protein C replacement in the treatment of meningococcemia-associated purpura fulminans.
Rivard
G E
GE
Division of Hematology-Oncology, Hôpital Sainte-Justine, Université de Montréal, Québec, Canada.
David
M
M
Farrell
C
C
Schwarz
H P
HP
D000293:Adolescent; D016470:Bacteremia / Q000150:complications* / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy* / Q000209:etiology*; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008589:Meningococcal Infections / Q000150:complications* / Q000188:drug therapy*; D010865:Pilot Projects; D011446:Prospective Studies; D011486:Protein C / Q000008:administration & dosage / Q000627:therapeutic use*; D011693:Purpura / Q000188:drug therapy / Q000209:etiology
D011486:Protein C