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

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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