Replacement therapy with a purified protein C concentrate during initiation of oral anticoagulation in severe protein C congenital deficiency

Thromb Haemost. 1993 Aug 2;70(2):247-9.

Abstract

The case of an adult patient with moderately severe protein C deficiency (antigen 16%, activity 12%) is reported. Both parents had protein C levels compatible with heterozygous deficiency. Unlike other reported cases of severe protein C deficiency in adults, the onset of thrombotic symptoms occurred at 1 month of age; however, a symptom-free period until age 17 followed. Replacement therapy with a monoclonal antibody purified protein C concentrate was carried out during the initiation of oral anticoagulation after a course of i.v. heparin for deep vein thrombosis. The administration of the concentrate allowed maintenance of protein C above 50% until a stable therapeutic anticoagulation level could be obtained. This was reached within a short time, thus allowing safe administration of a loading dose of warfarin. We conclude that this approach to the prevention of skin necrosis seems more rapid and safer than previous schedules of oral anticoagulation in protein C-deficient patients.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Oral
  • Adult
  • Aged
  • Combined Modality Therapy
  • Female
  • Heparin / therapeutic use
  • Homozygote
  • Humans
  • Infusions, Intravenous
  • Male
  • Middle Aged
  • Pedigree
  • Protein C / administration & dosage
  • Protein C / genetics
  • Protein C / therapeutic use*
  • Protein C Deficiency*
  • Recombinant Proteins / therapeutic use
  • Thrombolytic Therapy
  • Thrombophlebitis / drug therapy
  • Thrombophlebitis / genetics
  • Thrombophlebitis / therapy*
  • Tissue Plasminogen Activator / therapeutic use
  • Urokinase-Type Plasminogen Activator / therapeutic use
  • Warfarin / therapeutic use

Substances

  • Protein C
  • Recombinant Proteins
  • Warfarin
  • Heparin
  • Tissue Plasminogen Activator
  • Urokinase-Type Plasminogen Activator