2025 Volume 39 Issue 5 Pages 818-825
A 57-year-old man had elevated hepatic enzymes and esophageal varices on routine screening. CT and MRCP revealed a middle bile duct stricture with marked wall thickening and extrahepatic portal vein occlusion. EUS showed hypervascularity in the bile duct wall and surrounding collateral veins, indicating bile duct varices secondary to extrahepatic portal vein occlusion. Initially, the cause of the portal vein thrombosis was unclear, and endoscopic injection sclerotherapy for esophageal varices was only minimally effective. Anticoagulation therapy also failed to improve the thrombosis, and the patient was followed conservatively. After three and a half years, the thrombosis extended to the superior mesenteric and splenic veins, prompting reevaluation. Genetic testing revealed a JAK2V617F mutation, and bone marrow examination confirmed essential thrombocythemia. A hypercoagulable state from the JAK2 mutation and essential thrombocythemia likely contributed to portal vein thrombosis and bile duct varices. This case suggests the importance of evaluating myeloproliferative disorders, performing JAK2 testing, and collaborating with hematologists in unexplained extrahepatic portal vein occlusion.
