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Options for management of SCPCD

What are some of the different options for treating Severe Congenital
Protein C Deficiency?

Treating the manifestations of Severe Congenital Protein C Deficiency (SCPCD) usually involves anti-coagulation and/or protein C (PC) replacement, depending on the circumstances.1 Due to the small number of cases, long-term management of SCPCD is poorly understood.2,3 Evidence is usually derived from case reports, case series or retrospective clinical trials; all of which have limitations in terms of comparing results and extrapolating to a wider population.

The acute management of SCPCD involves PC replacement. Anticoagulants may be added concomitantly or during the transition to long-term management. Maintenance can be achieved through, oral anticoagulants or a combination of oral anticoagulants and PC replacement. Curative treatment has been achieved with liver transplantation.4,5

PC replacement is an established method for treating Purpura Fulminans (PF) and warfarin-induced skin necrosis, and can also be used for prophylaxis in patients with SCPCD.5 Replacement of PC can be achieved with infusions of fresh frozen plasma (FFP), cryoprecipitate, PC-rich prothrombin-complex concentrate or PC concentrate.2,4-6 FFP contains all plasma components (water, electrolytes, albumin immunoglobulins coagulation factors and factors of the complement system) including PC in a non-concentrated form.5,7 Prothrombin complex concentrates can consist of a combination of factors, such as factors II, VII, IX, PC and protein S.8

Guidelines, published by the American College of Chest Physicians for antithrombotic therapy in neonates and children, recommend the administration of either FFP or PC concentrate, when available, until the clinical lesions resolve.9

PC replacement therapy temporarily increases a patient’s PC levels, slowing down the clotting process and preventing thrombotic events associated with excess blood coagulation.11

Selected treatment options*

 

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References:

  1. Pescatore SL. Clinical management of protein C deficiency. Expert opinion on pharmacotherapy. 2001;2(3):431–439.

  2. Mathias M, et al. Subcutaneous administration of protein C concentrate. Pediatric hematology and oncology. 2004;21(6):549–554.

  3. Monagle K, et al. Long-term follow-up of homozygote protein C deficiency after multimodal therapy. Journal of pediatric hematology/oncology. 2014;36(7):e452–455.

  4. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

  5. Kroiss S, Albisetti M. Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency. Biologics: Targets & Therapy. 2010;4:51–60.

  6. Knoebl PN. Human protein C concentrates for replacement therapy in congenital and acquired protein C deficiency. Drugs of Today. 2008;44(6):429–441.

  7. Mercer University School of Medicine. The Internet Pathology Laboratory for Medical Education. Blood Products. Available at: http://library.med.utah.edu/WebPath/TUTORIAL/BLDBANK/BBPROD.html; Last accessed: May 2018.

  8. Franchini M, Lippi G. Prothrombin complex concentrates: an update. Blood Transfus. 2010;8(3):149–154.

  9. Monagle P, et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College
    of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141(2 Suppl):e737S-e801S.

  10. EMA. CEPROTIN: EPAR summary for the public. 2007.

  11. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  12. Stewart A. Warfarin-induced skin necrosis treated with protein C concentrate (human). American Journal of Health-System Pharmacy. 2010;67(11):901–904.

  13. Hermans C, et al. Dabigatran etexilate (Pradaxa®) for preventing warfarin-induced skin necrosis in a patient with severe protein C deficiency. Thrombosis and haemostasis. 2012;107(6):1189–1191.

  14. Beyer-Vestendorf J, et al. Prevention of thrombaembolic complications in patients with superficial-vein thrombosis given rivaroxaban or fondaparinux: the open-label, randomised, non-inferiority SURPRISE phase 3b trial. Lancet Haematol. 2017;4(3):e105-e113.

  15. Bauer KA. Fondaparinux: Dosing and adverse effects. Available at: https://www.uptodate.com/contents/fondaparinux-dosing-and-adverse-effects; Last accessed: September 2018.

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