Protein S deficiency: a clinical perspective

Haemophilia. 2008 Nov;14(6):1222-8. doi: 10.1111/j.1365-2516.2008.01775.x. Epub 2008 May 7.

Abstract

Protein S (PS) is an extensively studied protein with an important function in the down-regulation of thrombin generation. Because of the presence of a pseudogene and two different forms of PS in plasma, a bound and a free form, it is one of the most difficult thrombophilias to study. A deficiency of PS predisposes subjects to (recurrent) venous thromboembolism (VTE) and foetal loss. However, the conundrum of diagnosing PS deficiency has led to conflicting reports of PS as a risk factor for VTE. In this review, we aim to present a clinical perspective of PS deficiency.

Publication types

  • Review

MeSH terms

  • Adult
  • Age of Onset
  • Anticoagulants / administration & dosage
  • Blood Coagulation Tests
  • Blood Proteins / genetics*
  • Contraceptives, Oral, Hormonal
  • Contraindications
  • Estrogen Replacement Therapy
  • False Positive Reactions
  • Female
  • Heterozygote
  • Humans
  • Immunoassay
  • Infant, Newborn
  • Male
  • Middle Aged
  • Mutation
  • Polymerase Chain Reaction / methods
  • Pregnancy
  • Pregnancy Complications, Hematologic / genetics*
  • Pregnancy Trimesters
  • Protein S / genetics
  • Protein S / metabolism*
  • Protein S Deficiency / diagnosis
  • Protein S Deficiency / genetics*
  • Protein S Deficiency / physiopathology
  • Recurrence
  • Risk Factors
  • Thrombophilia / genetics
  • Venous Thromboembolism / drug therapy
  • Venous Thromboembolism / etiology*

Substances

  • Anticoagulants
  • Blood Proteins
  • Contraceptives, Oral, Hormonal
  • PROS1 protein, human
  • Protein S