Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiency

J. Conard, K. A. Bauer, A. Gruber, J. H. Griffin, H. P. Schwarz, M. H. Horellou -, M. M. Samama, R. D. Rosenberg

Research output: Contribution to journalArticle

49 Scopus citations

Abstract

Homozygous or doubly 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.

Original languageEnglish (US)
Pages (from-to)1159-1164
Number of pages6
JournalBlood
Volume82
Issue number4
DOIs
StatePublished - 1993

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Conard, J., Bauer, K. A., Gruber, A., Griffin, J. H., Schwarz, H. P., Horellou -, M. H., Samama, M. M., & Rosenberg, R. D. (1993). Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiency. Blood, 82(4), 1159-1164. https://doi.org/10.1182/blood.v82.4.1159.1159