title: 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.affiliation: Istituto Semeiotica Medica, Università Cattolica, Rome, Italy.
authors.forename: V
authors.initials: V
authors.lastname: De Stefano
authors.forename: S
authors.initials: S
authors.lastname: Mastrangelo
authors.forename: H P
authors.initials: HP
authors.lastname: Schwarz
authors.forename: P
authors.initials: P
authors.lastname: Pola
authors.forename: R
authors.initials: R
authors.lastname: Flore
authors.forename: B
authors.initials: B
authors.lastname: Bizzi
authors.forename: G
authors.initials: G
authors.lastname: Leone
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D006493:Heparin / Q000627:therapeutic use; D006720:Homozygote; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011486:Protein C / Q000008:administration & dosage / Q000235:genetics / Q000627:therapeutic use*; D020151:Protein C Deficiency; D011994:Recombinant Proteins / Q000627:therapeutic use; D015912:Thrombolytic Therapy; D013924:Thrombophlebitis / Q000188:drug therapy / Q000235:genetics / Q000628:therapy*; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use; D014568:Urokinase-Type Plasminogen Activator / Q000627:therapeutic use; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D011486:Protein C; D011994:Recombinant Proteins; D014859:Warfarin; D006493:Heparin; D010959:Tissue Plasminogen Activator; D014568:Urokinase-Type Plasminogen Activator