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Protein Deficiency C: Causes, Symptoms, Diagnosis, Treatment
Last reviewed: 23.04.2024
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[Epidemiology
The prevalence of heterozygous deficiency of plasma protein C is from 0.2 to 0.5%; About 75% of people with this anomaly have a history of venous thromboembolism (50% under the age of 50 years). Homozygous or double heterozygous deficiency leads to fulminant purpura of newborns, i.e. To severe neonatal DVS. The acquired deficit is manifested in patients with liver disease, ICE, during chemotherapy for cancer (including L-asparaginase) and in warfarin therapy.
Diagnostics of the protein deficiency C
The diagnosis is based on the determination of protein C antigen and functional studies of plasma coagulation (the degree of increase in the partial thromboplastin time of normal plasma, the use of plasma without protein C with the addition of plasma of the patient and snake venom).
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Treatment of the protein deficiency C
Patients with symptoms of thrombosis require anticoagulant therapy with unfractionated or low-molecular-weight heparin followed by the administration of warfarin. The use of vitamin K antagonists, warfarin as initial therapy, sometimes causes thrombotic skin infarction, induced by a decrease in the level of vitamin K-dependent protein C, which occurs before the decrease in the content of the remaining vitamin K-dependent clotting factors occurs. Fulminant purpura of newborns without carrying out substitution therapy with protein C (using normal plasma or factor concentrate) and anticoagulant therapy with heparin leads to death.