Portal, splenic and mesenteric vein thrombosis in a patient double heterozygous for factor V Leiden and prothrombin G20210A mutation

We herein report a 56-year-old man who presented with abdominal pain, diarrhea and a 22-kg-weight loss over 4 months. He was on acenocoumarol treatment because of portal, splenic and mesenteric vein thrombosis (PSMVT) 3 months before, with admission international normalized ratio (INR):1.6. Doppler ultrasonography and helical computerized tomographic scan of the abdomen showed complete thrombosis of the extrahepatic portal vein extending into the superior mesenteric vein and splenic vein. The manifestation of thrombosis was in the absence of provocative stimuli or local cause. The patient had a negative history of venous thromboembolism. Thrombophilia workup revealed double heterozygosity for factor V Leiden and prothrombin G20210A mutation. He was immediately started with intravenous unfractionated heparin, followed by oral anticoagulation with target INR 2-3. Five days after a Doppler examination showed significant improvement in the flow within the portal vein, and a computerized tomographic scan of the abdomen 1 month later showed extensive recanalization of the portal venous system. The patient is now 36 months out from the second PSMVT episode and is doing well although maintaining oral lifelong anticoagulation. The case is of particular interest in that PSMVT was the first manifestation of this combined disorder. We conclude that all patients presenting with unexplained PSMVT should be investigated for the presence of a hypercoagulable state. Anticoagulation should be considered in all patients with this diagnosis and should be a lifelong therapy in those with an underlying thrombophilia. Blood Coagul Fibrinolysis 20:722-725 (C) 2009 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.