Deep Vein and Portal Vein Thrombosis in a Patient with Protein C Deficiency: Report of a Case

Abstract

A 34-year-old man (174 cm in height and 77kg in weight) consulted our hospital because of the swelling of his left lower leg on September in 2014. Contrast enhanced tomography revealed thrombus in the left popliteal vein. There was no family history of thrombotic disease. Obesity and taking a sitting position for a long time were considered to be the cause of deep vein thrombosis. He was treated with 7.5 mg of fondaparinux for 7 days and 3mg of warfarin for 6 months.

Seven months after the discontinuation of warfarin he had abdominal discomfort and consulted our hospital again. Upon arrival he was in distress due to abdominal pain but on physical examination, his abdomen was soft and there was no rebound tenderness. Abdominal computed tomography scan revealed the portal vein thrombosis and segmental edema of small intestine. Because intestinal blood flow was likely to be intact, 240,000 units of urokinase and 20,000 units of heparin were administered systemically for 7 days. Laboratory examination on admission revealed normal protein S, antithrombin III, plasminogen levels. Protein C antigen and activity were 41% and 54 % respectively and inherited Protein C deficiency was strongly suggested. However, this patient didn’t want to take further studies including gene genetics. The patient recovered fully and was discharged on the 40th day after the admission while taking 3mg of warfarin to maintain the prothrombin international normalized ratio (PT-INR) within to 2.0. He is well and has no recurrence of venous thrombosis at follow-up 6 months after discharge.

Combination of deep vein and portal vein thrombosis due to Protein C deficiency is rare. Only four case reports similar to our case were found in the Japanese literature from 2004 to 2016. If a patient has unknown recurrent venous thrombosis, specific coagulation tests including Protein C, Protein S and Antithrombin III deficiency should be performed.