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. 2010 Mar 24:4:51-60.
doi: 10.2147/btt.s3014.

Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency

Sabine Kroiss  1 , Manuela Albisetti
Affiliations

Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency

Sabine Kroiss et al. Biologics. .

Abstract

Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulability state that usually presents at birth with purpura fulminans and/or severe venous and arterial thrombosis. Recurrent thrombotic events are commonly seen. From the 1990's, several virus-inactivated human protein C concentrates have been developed. These concentrates currently constitute the therapy of choice for the treatment and prevention of clinical manifestations of severe congenital protein C deficiency. This review summarizes the available information on the use of human protein C concentrates in patients with severe congenital protein C deficiency.

Keywords: Congenital protein C deficiency; protein C concentrate; purpura fulminans.

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