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Protein C Deficiency

Shilpa Das, MS, DO and Kyle J. Fletke, MD Reviewed 06/2022
 


BASICS

A rare heritable disorder or acquired risk factor causing increased risk of blood clots due to decreased anticoagulation. Symptoms range from asymptomatic presentation to recurrent venous throm...

DIAGNOSIS

Can be suspected with increased history as noted below 

HISTORY

  • Recurrent VTE

  • VTE at <40 years of age

  • Thrombosis in unusual locations (e.g., mesentery, sagittal sinus, portal vein)

  • Thrombosis ...

TREATMENT

Treat active thrombosis; follow-up 

GENERAL MEASURES

  • Individuals with PCD should be educated regarding signs and symptoms of VTE.

  • Women with any of the following history should avoid estradiol-...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Warfarin requires periodic monitoring of the INR to maintain a range of 2 to 3 (monthly, after initial stabilization).

  • LMWH is the treatment of c...

REFERENCES

1
Baglin  T, Gray  E, Greaves  M, et al; and British Committee for Standards in Haematology. Clinical guidelines for testing for heritable thrombophilia. Br J Haemato...

CODES

ICD10

D68.59 Other primary thrombophilia 

SNOMED

  • 76407009 Protein C deficiency disease (disorder)

  • 439002002 Thrombophilia due to acquired protein C deficiency

  • 441101007 heterozygous protein C deficie...

CLINICAL PEARLS

  • Screening of asymptomatic family members is not justified with the possible exception of females of childbearing age with no previous history of pregnancy complications.

  • Asymptomatic pa...

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