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Protein C Deficiency

Donald J. Fahey-Ahrndt, MD and James E. Hougas, III, MD, FAAFP Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Protein C is a vitamin K–dependent factor synthesized, in an inactive form, by the liver.

  • Activated protein C inhibits generation of thrombin by inactivating factors Va and VIIIa, usi...

DIAGNOSIS

HISTORY

  • Recurrent VTE

  • VTE at <40 years of age

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

  • Family history of thrombosis or spontaneous abortion

  • Often has othe...

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
Chan YC, Valenti D, Mansfield AO, et al. Warfarin induced skin necrosis. Br J Surg.  2000;87(3):266–272. [View Abstract on OvidMedline]
2
Baglin T, Gray E, Greaves M, et al; and British...

CODES

ICD10

D68.59 Other primary thrombophilia 

ICD9

289.81 Primary hypercoagulable state 

SNOMED

  • 76407009 Protein C deficiency disease (disorder)

  • 439002002 Thrombophilia due to acquired protein C deficienc...

CLINICAL PEARLS

  • Testing for heritable causes of thrombophilia in unselected patients presenting with a first episode of VTE (provoked or unprovoked) is not indicated.

  • Screening, and therefore prophylac...

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