Abstract

INTRODUCTION: Thrombocytopenia is normally a sequela of decompensated cirrhosis. Essential thrombocythaemia (ET), by contrast, is a myeloproliferative disorder involving an elevated platelet count that can manifest as thromboses of microvasculature. The rare concurrence of these two conditions is challenging for providers in that a normal platelet count may not be a proper indication of an adequate synthetic liver function, as a baseline value is unknown. CASE DESCRIPTION/METHODS: 66-year-old female with a history of ET and untreated chronic hepatitis B and C viral infections, who presented with intermittent left upper quadrant pain. On physical exam, the patient’s vital signs were within normal limits, and abdomen was non-tender on palpation. A complete metabolic panel was significant for elevated aminotransferases, with AST 78, ALT 78, and an alkaline phosphatase of 164. Additionally, a complete blood count was significant for thrombocytosis to 879 and hemoglobin of 10.6. Magnetic resonance imaging of the abdomen showed evidence of cirrhosis with sequelae of portal hypertension, including splenomegaly and upper abdominal varices, which were also seen on computed tomography of abdomen. She was diagnosed with new-onset cirrhosis secondary to chronic untreated viral hepatitis. Thrombopoietin (TPO) levels were not assessed as platelet count was markedly elevated. The patient was continued on her home hydroxyurea for continued management of ET. DISCUSSION: Platelet count is an important measure for patients with cirrhosis. Calculation like the AST to Platelet Ratio Index (APRI) score is used to predict disease severity. Assessing platelet production as an indicator of synthetic function in cirrhosis becomes difficult when treating patients with known ET. Prior cases have suggested that the thrombocytosis in ET may be attributed to an abnormality of the normal feedback inhibition responsible for platelet turnover. TPO levels are generally normal in ET and are notably reduced in decompensated cirrhosis. Providers, therefore, should consider direct measurement of TPO levels in patients with normal platelet counts who are diagnosed with ET and cirrhosis in order to assess whether synthetic liver function is impacted by liver disease. Furthermore, patients with cirrhosis have an increased risk of portal vein thrombosis (PVT); given that patients with both cirrhosis and ET may have an even higher risk of PVT, providers may consider prophylactically anticoagulating these patients to prevent such a complication.

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