A Case of Protein S Deficiency Presented with Superior Mesenteric and Portal Vein Thrombosis

Abstract

A 60-year-old man suffering from vomiting and abdominal pain was diagnosed with ileus. Computed tomography showed a thrombus in the superior mesenteric and portal vein. Laboratory tests showed an elevated D-dimer of 10. 0 μg/ml. Protein S was markedly decreased in activity (<10%) and antigen (46%). The venous thrombus disappeared with anticoagulation therapy and symptoms were relieved. The patient was discharged 45 days after admission, but hospitalized about 5 months later due to ileus recurrence without thrombosis. A small intestine series showed localized stenosis in the ileum. Laparotomy showed ileal stenosis about 60 cm proximal to the ileocolic junction. We resected the lesion with end-to-end anastomosis. Histopathological findings showed a penetrating ulcer scar with acute inflammatory cell infiltration and organized mesenteric vein thrombi in the lesion. The patient had no underlying disease that would have caused thrombosis, and decreaed protein S activity was confirmed in his 2 daughters. We concluded that this was a rare case of superior mesenteric and portal vein thrombosis due to congenital protein S deficiency.