Activation of the protein C pathway in hereditary thrombophilia

Elena M. Faioni, Franca Franchi, Daniela Asti, Pier Mannuccio Mannucci

Research output: Contribution to journalArticlepeer-review


Levels of free activated protein C are a measure of the activation of the protein C pathway in vivo. The aim of this study was to establish if the protein C pathway is triggered in familial thrombophilia and if activated protein C levels correlate with type of defect or symptoms. We measured activated protein C in 133 patients with a deficiency of antithrombin (n = 31), protein C (n = 24) or protein S (n = 27) or with resistance to activated protein C (n = 51). Levels of activated protein C were evaluated also in 97 healthy individuals. Results indicate that the levels of activated protein C are higher in patients who have experienced a thrombotic event than in patients who have not and that 71% of patients with levels of activated protein C above the normal reference range had had a venous thromboembolic event. We conclude that the protein C pathway is triggered in patients with thrombophilia and that in symptomatic patients, activated protein C levels are increased and may reflect heightened coagulation activation and scavenging through the protein C pathway.

Original languageEnglish
Pages (from-to)557-560
Number of pages4
JournalThrombosis and Haemostasis
Issue number4
Publication statusPublished - Oct 1998

ASJC Scopus subject areas

  • Hematology


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