Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis

Abstract

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. Key point Budd-Chiari syndrome is due to the obstruction of the hepatic efferent outflow tract from either the hepatic veins to where the inferior vena cava meets the right atrium. Its pathogenesis is due to the decreased outflow of the liver, leading to increase in pressure, stasis and later on damage induced by hypoxia and thrombus in the portal veins. Citation: Majid Z, Tasneem AA, Luck NH, Ul Haque MM, Mandhwani RK, Laeeq SM, et al. Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis Immunopathol Persa. 2018;4(1):e04. DOI: 10.15171/ipp.2018.04.