2023 Volume 45 Issue 2 Pages 167-174
A 37-year-old male presented to the emergency department of our hospital with a frontal headache and repeated tonic–clonic convulsions, having a convulsion lasting a few minutes the day before. We administered general anesthesia to control status epilepticus. A CT scan revealed cerebral venous sinus thrombosis (CVT) in the superior sagittal sinus. His father had a history of deep venous thrombosis. The protein C activity level was markedly reduced. The patient was diagnosed with protein C deficiency, and genetic analysis revealed a heterozygous mutation at exon7 c.631C>T, p.Arg211Trp (CGG→TGG) on the protein C gene. We started anticoagulation therapy with continuous intravenous heparin injection immediately, and warfarin was administered a few days later. After administration of warfarin, D-dimer levels increased rapidly and he developed pulmonary embolism. We administered an additional intravenous bolus of 3000 units of heparin and increased the dosage of warfarin gradually. The thrombus disappeared, and he remains fully recovered with no adverse events for two years. The risk of developing a transient hypercoagulable state possibly leading to microthrombi and skin necrosis has been reported in patients with protein C deficiency due to the rapid saturation of warfarin. Frequent D-dimer measurement should be considered in the initial phase of warfarin therapy for CVT caused by protein C deficiency.
