Portal Vein Thrombosis and Budd-Chiari Syndrome as the Inital Symptom of Polycythemia Vera and Hyperhomocysteinemia

• Main Authors: Behshad Pazooki, Hanieh Radkhah, Alborz Sherafat
• Format: Article
• Language: English
• Published: Tehran University of Medical Sciences 2019-08-01
• Series: Case Reports in Clinical Practice
• Subjects: Portal vein thrombosis; Polycythemia vera; Budd-Chiari syndrome; Hyperhomocysteinemia
• Online Access: https://crcp.tums.ac.ir/index.php/crcp/article/view/244

Portal Vein Thrombosis (PVT), commonly associated with cirrhosis of liver and thrombophilia, is one of the causes of severe abdominal pain. In the absence of non-cirrhotic non-malignant extrahepatic portal vein thrombosis, Myeloproliferative Disease (MPD) and an underlying thrombotic disorder should always be suspected and investigated. Hyperhomocysteinemia has been well-documented to increase the risk of arterial thrombotic events, peripheral arterial disease, and stroke. It is also a risk factor for deep-vein thrombosis. In the general population, association with portal vein thrombosis is very unusual, and only a few cases have been reported. We describe a case of Polycythemia Vera (PV) and hyperhomocysteinemia presenting with severe abdominal pain due to portal vein thrombosis. The patient underwent phlebotomy and was prescribed life-long anticoagulant, aspirin, vitamin B6, vitamin B12, and folic acid, then referred to a hematologist.