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[Protein C deficiency--a risk factor for venous thrombosis]
Klinische Wochenschrift
|October 15, 1984
Summary
This study identifies protein C deficiency in a young female patient with recurrent thrombosis and pulmonary embolism. Protein C deficiency increases risk for coumarin-induced skin necrosis, preventable with concurrent heparin use.
Area of Science:
- Biochemistry
- Hematology
- Genetics
Background:
- Protein C is a vital vitamin K-dependent anticoagulant synthesized in the liver.
- Activated protein C regulates coagulation by inactivating factors V and VIII.
Observation:
- A young female patient presented with recurrent deep vein thrombosis, pulmonary embolism, and superficial thrombophlebitis.
- Reduced protein C antigen and activity levels were detected in the patient's plasma.
- Protein C deficiency was identified in her father, sister, and son, indicating hereditary transmission.
Findings:
- The patient experienced coumarin-induced skin necrosis during anticoagulant therapy with Marcumar.
- This complication is strongly associated with protein C deficiency.
- Concurrent administration of heparin during the initial Marcumar adjustment period prevented skin necrosis.
Implications:
- Protein C deficiency poses a significant risk for severe thrombotic events.
- Screening for protein C deficiency is crucial in patients with unexplained thrombosis.
- Careful anticoagulant management, potentially involving heparin bridging, is essential for patients with protein C deficiency to prevent complications like skin necrosis.