Clinical Pathology: Hematology & Coagulation, Transfusion Medicine

• Severe protein C deficiency (homozygous protein C deficiency) can present as life-threatening purpura fulminans and disseminated intravascular coagulation (DIC).

• Purpura fulminans is characterized by ecchymotic skin lesions that spontaneously become necrotic. Purpura fulminans is often associated with DIC.

• Protein C is a vitamin K–dependent natural anticoagulant. Activated protein C, with its cofactor protein S, inactivates factors VIIIa and Va.

• Heterozygous hereditary protein C deficiency is seen in 3% of patients with their first venous thromboembolism (VTE) and in only 0.14% to 0.5% of the general population.

• Protein C deficiency increases the risk for VTE by seven-fold.

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