title: Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency.
abstract: Severe genetic protein C deficiency is rare and is associated with severe, often fatal thrombosis. The authors report the use of recombinant activated protein C (APC) to treat an episode of purpura fulminans (PF) in a teenage girl with severe protein C deficiency who had developed anaphylaxis to fresh-frozen plasma that was given in the past to treat recurrent episodes of PF. Concomitant with an infusion of APC, 20 microg/kg/h for 10 hours, a d-dimer level that was markedly positive (6,450 ng/mL) prior to the onset of PF decreased to 847 ng/mL following the APC. The teenager was treated with heparin along with warfarin for four days until the INR was more than 3.5 and the d-dimer level was less than 230 ng/mL. At the end of the APC infusion all skin lesions of PF were resolved. There were no adverse reactions to APC. APC was safe and effective for treatment of PF in severe genetic protein C deficiency.
authors.affiliation: Mountain States Regional Hemophilia and Thrombosis Center, P.O. Box 6507, Mail Stop F-416, Aurora, CO 80045-0507, USA. marilyn.manco-johnson@uchsc.edu
authors.forename: Marilyn J
authors.initials: MJ
authors.lastname: Manco-Johnson
authors.forename: Rhonda
authors.initials: R
authors.lastname: Knapp-Clevenger
mesh_terms: D000293:Adolescent; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis / Q000187:drug effects; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D011695:IgA Vasculitis / Q000188:drug therapy* / Q000209:etiology; D019934:International Normalized Ratio; D011486:Protein C / Q000008:administration & dosage*; D020151:Protein C Deficiency / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D011994:Recombinant Proteins; D012074:Remission Induction; D016896:Treatment Outcome; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; D011486:Protein C; D011994:Recombinant Proteins; C036309:fibrin fragment D; D014859:Warfarin; D006493:Heparin