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

Zain Majid, Abbas Ali Tasneem, Nasir Hassan Luck, Muhammad Manzoor Ul Haque, Rajesh Kumar Mandhwani, Syed Mudassir Laeeq, Farina Mohammad Hanif, Ghulamullah Lail


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.



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