Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiency
| Autor: | J, Conard, K A, Bauer, A, Gruber, J H, Griffin, H P, Schwarz, M H, Horellou, M M, Samama, R D, Rosenberg |
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| Rok vydání: | 1993 |
| Předmět: | |
| Zdroj: | Blood. 82:1159-1164 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v82.4.1159.bloodjournal8241159 |
| Popis: | Homozygous or double heterozygous protein-C deficiency can present at birth with purpura fulminans or later in life with venous thrombosis. Two homozygous patients who had previously sustained thrombotic episodes were investigated at a time when they were asymptomatic and not receiving antithrombotic therapy. The plasma levels of protein-C antigen and activity in both individuals were approximately 20% of normal. We administered a highly purified plasma-derived protein C concentrate to these individuals and monitored levels of several markers of in vivo coagulation activation. Assays for protein-C activation (activated protein C and protein C activation peptide) showed a sustained increase from reduced baseline levels, whereas thrombin generation (as measured by prothrombin fragment F1 + 2) gradually decreased over about 24 hours into the normal range. These investigations provide direct evidence that protein C is converted to activated protein C in vivo, and that the protein-C anticoagulant pathway is a tonically active mechanism in the regulation of hemostatic system activation in humans. |
| Databáze: | OpenAIRE |
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