Protein C Deficiency Treatment & Management

  • Author: Adam Cuker; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Oct 26, 2011
 

Medical Care

As noted earlier, 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 in patients with protein C deficiency is managed in much the same way as it is for patients with VTE due to other causes (see Further Reading). Because the risk of recurrent VTE in protein C – deficient patients may be as high as 60%,[20] 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.[58]

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.[59, 60, 61]

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.[62] 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.[63]

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Consultations

Consultation with a hematologist is warranted for the care of patients with congenital protein C deficiency.

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Diet

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.

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Activity

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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Contributor Information and Disclosures
Author

Adam Cuker  MD, MS, Assistant Professor of Medicine, Assistant Professor of Pathology and Laboratory Medicine, University of Pennsylvania

Adam Cuker is a member of the following medical societies: American College of Physicians, American Society of Hematology, Hemophilia and Thrombosis Research Society, International Society on Thrombosis and Haemostasis, and National Hemophilia Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Eleanor S Pollak, MD  Associate Director of Special Coagulation, Associate Professor, Department of Pathology and Laboratory Medicine, Section of Hematology and Coagulation, University of Pennsylvania

Eleanor S Pollak, MD is a member of the following medical societies: American Society of Hematology, College of American Pathologists, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David Aboulafia, MD  Medical Director, Bailey-Boushay House, Clinical Professor, Department of Medicine, Division of Hematology, Attending Physician, Section of Hematology/Oncology, Virginia Mason Clinic; Investigator, Virginia Mason Community Clinic Oncology Program/SWOG

David Aboulafia, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Medical Directors Association, American Society of Hematology, Infectious Diseases Society of America, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Troy H Guthrie, Jr, MD  Director of Cancer Institute, Baptist Medical Center

Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

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The protein C pathway. APC = activated protein C; PC = protein C; S= protein S; T = thrombin; TM = thrombomodulin; Va = factor Va; VIII = factor VIIIa.
A patient with warfarin-induced skin necrosis.
A patient with neonatal purpura fulminans.
 
 
 
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