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Protein C Deficiency

Kyle J. Fletke, MD Reviewed 04/2024
 


BASICS

Protein C deficiency (PCD) is a rare heritable disorder or acquired risk factor resulting in a prothrombotic state. Symptoms range from an asymptomatic presentation to recurrent venous thromboe...

DIAGNOSIS

HISTORY

  • Recurrent VTE

  • VTE at <40 years of age

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

  • Thrombosis at a young age (<50 years)

  • Family history of thrombos...

TREATMENT

GENERAL MEASURES

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

  • Women with any of the following history should avoid estradiol-containing contraceptives and hormo...

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
Trait RC, Walker ID, Reitsma PH, et al. Prevalence of protein C deficiency in the healthy population. Thromb Haemost. 1995;73(1):87–93.
2
Baglin  T, Gray  E, Greaves  ...

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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