Protein C deficiency is a rare genetic trait that can lead to excessive blood clotting and thrombotic disease. It is associated with thrombosis in veins, but not arteries. Treatment options include anticoagulant drugs and supplemental protein C concentrates. Warfarin can induce protein C deficiency in some patients.
Protein C deficiency is a genetic trait that predisposes one to thrombotic disease, in which the blood clots too much, causing blood vessel blockages or embolism. Protein C deficiency is associated with thrombosis in the veins, but not in the arteries. The condition is very rare, affecting only 0.2 to 0.5% of the population.
Protein C is an anticoagulant, meaning it prevents blood from clotting or clotting. Of course, coagulation is a very important function of the blood, responsible for the healing of wounds. However, excessive clotting can cause problems of its own, including strokes and heart attacks. Therefore, the body is naturally equipped with anticoagulant regulators, one of the most important being protein C.
There are two types of protein C deficiency. In Type I, the patient does not produce enough protein, while in Type II, the protein does not work properly, interacting abnormally with other molecules. In most people with protein C deficiency, only one of the two genes responsible for protein C is dysfunctional, so there is still some functioning protein C in the blood.
Some people are born with two defective copies of the gene responsible for protein C, resulting in a complete deficiency of the protein. While this condition is extremely rare, it can lead to the development of purpura fulminans, also called purpura gangrenosa, in babies. This life-threatening condition is characterized by the formation of numerous small clots within blood vessels, excessive bleeding, tissue necrosis or death, fever, and low blood pressure. Homozygous protein C deficiency, in which both relevant genes are defective, should be treated very early with supplemental protein C concentrates.
Heterozygous protein C deficiency, in which only one gene is defective, is usually treated by giving anticoagulant drugs, such as aspirin, heparin, or warfarin. Such medications are usually taken regularly to prevent clotting, rather than in response to a thrombotic event. Anticoagulant medications are generally prescribed long-term for people with protein C deficiency, often for life.
In a related but extremely rare condition, the drug warfarin can induce protein C deficiency in patients who were not born with the condition. Although it is an anticoagulant, warfarin also inhibits protein C. In warfarin necrosis, excessive clotting prevents blood flow to the skin, resulting in lesions that eventually cause bleeding. Other anticoagulants do not have this problem, and are sometimes given along with warfarin at the start of warfarin treatment to prevent warfarin necrosis.
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