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Protein C deficiency
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Protein C deficiency

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Synopsis

Protein C is a normally occurring blood protein with anticoagulant features that is needed to prevent venous blood clots. Protein C deficiency creates a condition of high risk for developing venous thromboembolism in a common venous location, such as deep vein thrombosis of the legs or mesenteric veins in the colorectal region. Fewer than half of patients with protein C deficiency and thromboembolism exhibit other risk factors for thrombosis, such as pregnancy, surgery, hormonal therapy, postpartum state, advanced age, or immobility. Persons with mild protein C deficiency may be asymptomatic and never form blood clots.

The deficiency can be inherited or acquired. There are several variations of the inherited condition, or thrombophilia, which can range from asymptomatic to severe. Recurrent venous thromboembolism is typical of heterozygous protein C deficiency, subdivided into type I (decreased protein C) and type II (functionally defective protein C in normal amounts).

Acquired protein C deficiency can be caused by vitamin K deficiency, sepsis, bacterial infection, acute thrombosis, warfarin use, some chemotherapy agents, severe liver disease, or disseminated intravascular coagulation.

An association has been drawn between protein C deficiency and skin necrosis, liver disease, bone marrow transplant, renal failure, myocardial infarction, and ischemic stroke. Additional adverse effects may be due to anticoagulants and other medications used in the treatment of thromboembolism.

In neonates, a very low protein C level may contribute to purpura fulminans or result in a life-threatening condition of disseminated intravascular coagulation.

Patients in treatment for thromboembolism or complications due to protein C deficiency may be treated with anticoagulants. If heparin or warfarin are found to have problematic adverse effects, alternatives are fresh frozen plasma infusion or protein C concentrate. Patients with protein C deficiency without thromboembolism should be advised to avoid other risk factors for thrombotic events, such as hormonal therapy or prolonged periods of immobility. Also, patients should be advised about the risk for thromboembolism if pregnant, planning to become pregnant, or scheduling surgery.

Codes

ICD10CM:
D68.59 – Other primary thrombophilia

SNOMEDCT:
76407009 – Protein C deficiency disease

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 10/06/2017
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Protein C deficiency
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Protein C deficiency
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