Nickan Research Institute Immunopathologia Persa 2423-8015 4 1 2018 01 01 Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis e04 10.15171/ipp.2018.04 EN Zain Majid Abbas Ali Tasneem Nasir Hassan Luck Muhammad Manzoor Ul Haque Rajesh Kumar Mandhwani Syed Mudassir Laeeq Farina Muhammad Hanif Ghulamullah Lail Journal Article 10.15171/ipp.2018.04 2017 04 17 2017 06 29 A middle aged male presented with abdominal distension since one month. Further workup showed plasma hemoglobin of 18.1 g/dL with a high pack cell volume (PCV), raised urea, creatinine and disturbed liver function tests. Abdominal ultrasonography showed an enlarged caudate lobe with thrombi in the inferior vena cava while CT scan of abdomen confirmed the same findings and was suggestive of Budd-Chiari syndrome. Further workup was conducted to rule out other causes and to find out the possible cause of Budd-Chiari syndrome. A peripheral film was requested, which showed hyper-segmented neutrophils. Later on JAK2 mutation and thrombophilia profile was ordered, which was positive for JAK2 mutation. Even though the patient was started on low molecular weight heparin but he eventually passed away.