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Protein C Deficiency Follow-up

  • Author: Adam Cuker, MD, MS; Chief Editor: Perumal Thiagarajan, MD  more...
Updated: Jan 21, 2016


Venous thromboembolism prevention

Thromboprophylaxis should be considered for surgery, pregnancy and the puerperium, trauma, and prolonged air travel in individuals with heterozygous protein C deficiency, particularly if there is a strong family history of thrombosis. Similarly, estrogen-containing hormonal therapy should only be used in such patients after careful consideration of the thrombotic risk.

Warfarin-induced skin necrosis prevention

For patients with heterozygous protein C deficiency, the following is recommended in order to avoid the development of WISN:

  • Large loading doses of warfarin (>5 mg/d) should be avoided.
  • When warfarin is initiated, it should be overlapped with a parenteral anticoagulant such as unfractionated or low molecular weight heparin.
  • The parenteral anticoagulant should be continued for at least 5 days and until the international normalized ratio (INR) measurement has been 2.0 or greater for at least 2 days.

Patient Education

See the list below:

  • Patients with protein C deficiency should be advised of the presenting signs and symptoms of VTE.
  • Patients who are not maintained on anticoagulation should speak with their physician about thromboprophylaxis during events associated with an elevated risk of thrombosis such as surgery, trauma, immobilization, pregnancy, and the postpartum period.
  • Patients on warfarin should be advised of the importance of maintaining a regular diet and notifying their physician when changes to their medications have been made.
Contributor Information and Disclosures

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

Adam Cuker, MD, MS is a member of the following medical societies: American College of Physicians, American Society of Hematology, Anticoagulation Forum, Hemostasis and Thrombosis Research Society, International Society on Thrombosis and Haemostasis

Disclosure: Received consulting fee from Genzyme for consulting; Received consulting fee from Amgen for consulting; Received grant/research funds from T2 Biosystems for other; Received grant/research funds from Spark Therapeutics for other. .


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, National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Perumal Thiagarajan, MD Professor, Department of Pathology and Medicine, Baylor College of Medicine; Director, Transfusion Medicine and Hematology Laboratory, Michael E DeBakey Veterans Affairs Medical Center

Perumal Thiagarajan, MD is a member of the following medical societies: American College of Physicians, American Society for Clinical Investigation, Association of American Physicians, American Society for Biochemistry and Molecular Biology, American Heart Association, American Society of Hematology, Royal College of Physicians

Disclosure: Nothing to disclose.

Additional Contributors

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, AMDA - The Society for Post-Acute and Long-Term Care Medicine, American Society of Hematology, Infectious Diseases Society of America, Phi Beta Kappa

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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