Protein C substitution in sepsis-associated purpura fulminans.
OBJECTIVE
To assess the effect of protein C (PC) substitution on imminent peripheral necroses and overall outcome in patients with sepsis-associated purpura fulminans.


METHODS
Case series.


METHODS
Intensive care units of two university hospitals.


METHODS
A total of 12 patients with purpura fulminans, disseminated intravascular coagulation and imminent peripheral necroses in association with sepsis caused by Neisseria meningitidis (n = 5), Streptococcus pneumoniae (n = 2), Capnocytophaga canimorsus (n = 2), and Staphylococcus aureus (n = 1). In two patients, no pathogens were identified.


METHODS
Intravenous administration of PC concentrate (100 IU/kg every 6 hrs). In addition, antithrombin III substitution, antimicrobial therapy, hemodynamic support, and mechanical ventilation in all patients and hemodiafiltration in 10 patients.


RESULTS
After the onset of PC, progressive peripheral ischemia was reversed irrespective of the etiology of infection. Laboratory variables reflecting disseminated intravascular coagulation improved rapidly, although the recovery of the platelet count was retarded in the patients who subsequently died. No drug-related adverse events were noted. Amputations were necessary in two patients, and necrotic tips of fingers and toes were macerated in a third. The hospital mortality was 42%. Of the five lethal cases, two were caused by S. pneumoniae, one by N. meningitidis, one by C. canimorsus, and one by an unknown pathogen.


CONCLUSIONS
This article provides encouraging results on the use of PC substitution in meningococcal purpura and presents new data on the administration of this drug to patients with septic purpura caused by other bacterial species. By clinical judgment, PC limited the extent of tissue necrosis. The small number of patients does not allow for any conclusions on the potential effect of PC on mortality. A controlled and randomized study with a larger number of patients is needed before any recommendations can be given on the use of PC in sepsis-related purpura fulminans and shock.
Department of Medicine, Turku University Central Hospital, Finland. esa.rintala@satshp.fi
E
E
Rintala
M
M
Kauppila
O P
OP
Seppälä
L M
LM
Voipio-Pulkki
V
V
Pettilä
V
V
Rasi
P
P
Kotilainen
D018806:APACHE; D000293:Adolescent; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use*; D000990:Antithrombin III / Q000627:therapeutic use; D002097:C-Reactive Protein / Q000378:metabolism; D002206:Capnocytophaga; D003606:Dacarbazine / Q000097:blood*; D005260:Female; D005734:Gangrene / Q000601:surgery; D017583:Hemodiafiltration; D017052:Hospital Mortality; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000188:drug therapy; D007362:Intensive Care Units; D008297:Male; D008875:Middle Aged; D009345:Neisseria meningitidis; D011486:Protein C / Q000627:therapeutic use*; D012121:Respiration, Artificial; D018805:Sepsis / Q000150:complications* / Q000382:microbiology*; D015842:Serine Proteinase Inhibitors / Q000627:therapeutic use; D013296:Streptococcus pneumoniae; D016896:Treatment Outcome
D000925:Anticoagulants; D011486:Protein C; D015842:Serine Proteinase Inhibitors; D003606:Dacarbazine; D000990:Antithrombin III; D002097:C-Reactive Protein