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

Yoshio Uemura, Kenji Kobayashi, Taichi Koyama, Naofumi Kagara, Yosuke Seki, Taro Aoki, Toshisada Ukei, Keisuke Miyauchi and Tadashi Kaneko

Department of Surgery, Kinki Central Hospital

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 10.0μg/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.

Key words
protein S deficiency, mesenteric and portal vein thrombosis

Reprint requests
Yoshio Uemura Department of surgery Kinki, Central Hospital 3-1, Kurumazuka, Itami, 664-8533 JAPAN

Accepted
October 31, 2001

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