The devastating effect of protein c deficiency

Abstract

Protein C is a vitamin K-dependent anticoagulant protein synthesized in the liver. Upon activation, protein C inactivates coagulation factors Va and VIIIa, which are necessary for thrombin generation and factor X activation. Most patients with inherited protein C deficiency are heterozygous for a genetic defect that reduces protein C levels, activity, or both (ie, transmission is autosomal dominant). A number of acquired conditions also can reduce protein C levels, including acute thrombosis, disseminated intravascular coagulation (DIC), liver disease, vitamin K antagonist (VKA) anticoagulants, meningococcal infection, and others [1]. The following case illustrates a life threatening condition that Protein C deficiency can cause.