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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