European Journal of Pediatrics

, Volume 155, Issue 1, pp 20–25 | Cite as

Purpura fulminans in severe congenital protein C deficiency: Monitoring of treatment with protein C concentrate

  • F. -M. Müller
  • W. Ehrenthal
  • G. Hafner
  • D. Schranz


This report describes the successful use of protein C concentrate to treat severe purpura fulminans in a homozygous protein C-deficient infant for 8 months until oral anticoagulation was initiated. While fresh frozen plasma was previously used in such cases to replace protein C in the acute phase, the availability of a monoclonal antibody purified protein C concentrate now allows specific replacement of protein C, avoiding problems of fluid overload. An occlusive-hydrocolloid bandage proved to be effective in local treatment of skin lesions. D-dimer, fibrin monomer, thrombin-antithrombin complex and prothrombin fragment 1+2 were useful markers in monitoring and optimizing protein C replacement therapy.

Key Words

Purpura fulminans Severe congenital protein C deficiency Protein C concentrate Coagulation marker 



activated partial thromboplastin time


disseminated intravascular coagulation


prothrombin fragment 1+2


fibrin monomer


tissue plasminogen activator


thrombin-antithrombin complex


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Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • F. -M. Müller
    • 1
    • 3
  • W. Ehrenthal
    • 2
  • G. Hafner
    • 2
  • D. Schranz
    • 1
  1. 1.University Children's HospitalMainzGermany
  2. 2.Institute of Clinical ChemistryUniversity HospitalMainzGermany
  3. 3.University Children's HospitalAachenGermany

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