MPL-Positive Essential Thrombocytosis Presenting as Budd-Chiari Syndrome in a Middle-Aged Woman with an Initially Normal Platelet Count
  • Mohammad Ammad Ud Din
    Internal Medicine, Rochester General Hospital, Rochester, New York, USA
  • Hania Liaqat
    Internal Medicine, Rochester General Hospital, Rochester, New York, USA
  • Muhammad Osama
    Internal Medicine, Rochester General Hospital, Rochester, New York, USA

Keywords

Veno-occlusive disease, myeloproliferative disorders, Budd-Chiari syndrome, essential thrombocytosis, malignant hematology

Abstract

Budd-Chiari syndrome (BCS) results from an occlusion of the hepatic venous flow which in turn leads to portal hypertension causing ascites and other signs of liver dysfunction. Here, we present the case of a 43-year-old woman with recurrent ascites who was found to have BCS secondary to an inferior vena cava thrombosis extending into the hepatic veins. Although she had a normal platelet count on admission, additional laboratory investigations revealed an MPL mutation. She was discharged on anticoagulation with apixaban and later found to have thrombocytosis on repeat blood work, confirming the diagnosis of essential thrombocytosis, following which she was started on myelosuppressive therapy with hydroxyurea.

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    Published: 2021-12-17
    Issue: 2021: Vol 8 No 12 (view)

    How to cite:
    1.
    Ammad Ud Din M, Liaqat H, Osama M. MPL-Positive Essential Thrombocytosis Presenting as Budd-Chiari Syndrome in a Middle-Aged Woman with an Initially Normal Platelet Count. EJCRIM 2021;8 doi:10.12890/2021_003081.