Submitted: 17 Apr 2017
Accepted: 29 Jun 2017
ePublished: 05 Jul 2017
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Immunopathol Persa. 2018;4(1): e04.
doi: 10.15171/ipp.2018.04
  Abstract View: 4133
  PDF Download: 3629

Case Report

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

Zain Majid 1 * , Abbas Ali Tasneem 1, Nasir Hassan Luck 1, Muhammad Manzoor Ul Haque 1, Rajesh Kumar Mandhwani 1, Syed Mudassir Laeeq 1, Farina Muhammad Hanif 1, Ghulamullah Lail 1

1 Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan
Correspondence to Zain Majid Zain88@ hotmail.com


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. 
Keywords: Budd Chiari Syndrome, Polycythemia vera, JAK2 mutation, Inferior vena cava thrombosis
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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