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Protein S Deficiency

Boris Calderon, DO Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Protein S is a vitamin K–dependent glycoprotein, produced mainly in the liver that acts as a cofactor for protein C. It is also produced by megakaryocytes and endothelial cells.

  • Prote...

DIAGNOSIS

HISTORY

Inherited thrombophilias should be suspected in: 
  • Unprovoked VTE at age <50 years

  • VTE with a strong family history of VTE or known familial protein S deficiency

  • VTE in unusual sites a...

TREATMENT

GENERAL MEASURES

  • Routine anticoagulation for asymptomatic patients with protein S deficiency is not recommended.

  • Antithrombotic therapy recommendations for patients with protein S deficiency c...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Warfarin requires periodic (monthly after initial stabilization) monitoring of the INR.

  • Periodic measurement of INR to maintain a range of 2 to 3

  • ...

REFERENCES

1
Bick  RL. Prothrombin G20210A mutation, antithrombin, heparin cofactor II, protein C, and protein S defects. Hematol Oncol Clin North Am.  2003;17(1):9–36. [Vi...

ADDITIONAL READING

Ameku K, Higa M. Rivaroxaban Treatment for Warfarin-Refractory Thrombosis in a Patient with Hereditary Protein S Deficiency. Case Rep Hematol. 2018 Jan 23;2018:...

CODES

ICD10

D68.59 Other primary thrombophilia 

SNOMED

  • 1563006 protein S deficiency disease (disorder)

  • 439125003 Thrombophilia due to acquired protein S deficiency

  • 440988005 Heterozygous protein S deficien...

CLINICAL PEARLS

  • Asymptomatic patients with protein S deficiency do not require prophylactic anticoagulation because the risk of thrombosis is low; asymptomatic patients do not require anticoagulation.

  • ...

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