Protein C Deficiency Treatment & Management
- Author: Adam Cuker, MD, MS; Chief Editor: Perumal Thiagarajan, MD more...
A substantial proportion of individuals with protein C deficiency remain asymptomatic throughout life and require no specific therapy. However, thromboprophylaxis may be considered in such individuals, particularly if there is a strong family history of thrombosis, for situations associated with a high thrombotic risk such as pregnancy and the postpartum state, surgery, and trauma.
For those patients who do develop clinical manifestations of hereditary protein C deficiency, treatment depends on the particular clinical syndrome: venous thromboembolism (VTE), warfarin-induced skin necrosis (WISN), or neonatal purpura fulminans (NPF).
VTE in patients with protein C deficiency is managed in much the same way as it is for patients with VTE due to other causes. Because the risk of recurrent VTE in protein C–deficient patients may be as high as 60%, long-term anticoagulation is often recommended, particularly following a spontaneous thromboembolic event.
Warfarin-induced skin necrosis
WISN is a medical emergency that requires treatment as soon as it is recognized. Therapy consists of immediate discontinuation of warfarin, administration of vitamin K, and initiation of therapeutic doses of heparin. If the patient is protein C deficient, administration of exogenous protein C should be administered, either in the form of fresh frozen plasma (FFP) or, preferably, as purified protein C concentrate (Ceprotin) with the goal of expeditiously normalizing plasma protein C activity.
Neonatal purpura fulminans
Like WISN, NPF is a medical emergency that requires rapid normalization of plasma protein C activity. Although fresh frozen plasma has been used as a source of exogenous protein C in the treatment of NPF, frequent administration is required to maintain adequate plasma levels, thereby limiting its usefulness in this setting. Highly purified protein C concentrate (Ceprotin) represents an attractive alternative that does not subject patients to the high volume and protein load of fresh frozen plasma.[62, 63, 64]
After treatment of the acute phase of NPF, patients are transitioned to anticoagulation therapy, on which they must remain indefinitely. Warfarin may be used in this setting, provided that exogenous protein C is administered during its initiation in order to avoid the development of WISN. For patients with breakthrough thrombosis despite anticoagulation, protein C concentrate may be infused at home. A subcutaneous formulation of protein C requiring administration every 3 days has been used successfully in this context.
Living donor liver transplantations have been successfully performed in NPF, resulting in a permanent cure.
Consultation with a hematologist is warranted for the care of patients with congenital protein C deficiency.
Diet and Activity
There are no special dietary requirements for individuals with protein C deficiency. However, patients on warfarin should consume a steady diet and avoid large day-to-day fluctuations in the amount of vitamin K they ingest.
There are no specific restrictions with respect to physical activity that are recommended for individuals with protein C deficiency. All individuals should ambulate regularly during prolonged travel to reduce the risk of VTE. Patients on anticoagulation therapy should avoid contact sports to reduce the risk of trauma and major bleeding.
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