Format

Send to:

Choose Destination
See comment in PubMed Commons below
J Pediatr Hematol Oncol. 2004 Jan;26(1):25-7.

Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency.

Author information

  • 1Mountain States Regional Hemophilia and Thrombosis Center, P.O. Box 6507, Mail Stop F-416, Aurora, CO 80045-0507, USA. marilyn.manco-johnson@uchsc.edu

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.

PMID:
14707707
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Lippincott Williams & Wilkins
    Loading ...
    Write to the Help Desk