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

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.

• Authors: V De Stefano, S Mastrangelo, H P Schwarz, P Pola, R Flore, B Bizzi, G Leone
• Journal: Thrombosis and haemostasis (Thromb Haemost) Vol. 70 Issue 2 Pg. 247-9 (Aug 02 1993) ISSN: 0340-6245 [Print] Germany
• PMID: 8236128 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

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

Topics

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