Protein C deficiency in a Dutch family with thrombotic disease

Thromb Haemost. 1982 Aug 24;48(1):1-5.

Abstract

A rabbit antibody against human protein C was used for the quantitative estimation of protein C in plasma. In healthy individuals protein C antigen ranged from 0.65-1.45 U/ml. Plasma protein C antigen was found to be independent of either age or sex. Under influence of oral anticoagulant treatment the protein C antigen concentration decreased to 0.47 U/ml (at low intensity treatment) or 0.33 U/ml (at high intensity treatment). Using normal ranges of protein C and protein C/factor II and protein C/factor X ratios criteria were developed for the assessment of protein C deficiency. In a Dutch family with a history of thrombotic disease two members were found to have an isolated protein C deficiency, while a third one is suspected of protein C deficiency. In one case it was possible to confirm the diagnosis of suspected protein C deficiency during temporary withdrawal of the anticoagulant therapy.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Aging
  • Animals
  • Anticoagulants / therapeutic use
  • Blood Coagulation Disorders / complications
  • Blood Coagulation Disorders / epidemiology
  • Blood Coagulation Disorders / genetics*
  • Female
  • Glycoproteins* / analysis
  • Glycoproteins* / immunology
  • Humans
  • Immune Sera / pharmacology
  • Male
  • Middle Aged
  • Netherlands
  • Protein C
  • Pulmonary Embolism / etiology
  • Rabbits
  • Thrombophlebitis / epidemiology
  • Thrombophlebitis / etiology
  • Thrombophlebitis / genetics*

Substances

  • Anticoagulants
  • Glycoproteins
  • Immune Sera
  • Protein C