Eur J Pediatr. 1996 Jan;155(1):20-5.

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

Müller FM, Ehrenthal W, Hafner G, Schranz D.

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University Children's Hospital, Mainz, Germany.

Abstract

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. CONCLUSION: Diagnosis of protein C deficiency should be considered in a newborn with purpura fulminans. Early diagnosis and adequate replacement therapy is life-saving. Today, administration of protein C is the acute as well as long-term therapy of choice.

PMID:: 8750805; [PubMed - indexed for MEDLINE]

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Review Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiency. [Semin Thromb Hemost. 1995]
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Cited by 4 PubMed Central articles

Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency. [Biologics. 2010]
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency.
Kroiss S, Albisetti M. Biologics. 2010 Mar 24; 4:51-60. Epub 2010 Mar 24.
Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications. [Biologics. 2008]
Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications.
Knoebl PN. Biologics. 2008 Jun; 2(2):285-96.
Mice with a severe deficiency in protein C display prothrombotic and proinflammatory phenotypes and compromised maternal reproductive capabilities. [J Clin Invest. 2005]
Mice with a severe deficiency in protein C display prothrombotic and proinflammatory phenotypes and compromised maternal reproductive capabilities.
Lay AJ, Liang Z, Rosen ED, Castellino FJ. J Clin Invest. 2005 Jun; 115(6):1552-61. Epub 2005 May 5.

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