Abstract Number: PB2257
Meeting: ISTH 2020 Congress
Background: Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by excessive, clonal platelet production and increased risk of thrombotic complications.
Aims: Case report of a patient with ET complicated with chronic portal vein thrombosis (PVT) with liver cirrhosis, and treatment approach.
Methods: A 27-year-old man was admitted in the clinic for diagnostic work-up and therapy because of high platelet count (Plt) 908×109/l. He had 4 year history of hepatosplenomegaly, esophageal varices gr.III, and high Plt. The patient was recently confirmed with chronic PVT with collateral circulation, portal hypertension, liver cirrhosis ChildA, ascites, and was considered for TIPS. At the time of admission he was not taking any platelet reduction or anticoagulant therapy.
Results: Blood count showed Hb 148g/l, WBC 6.61×109/L, Plt 716×109/l; blood smear was normal. ET was diagnosed based on bone marrow biopsy which showed megakaryocytes proliferation without fibrosis, and positive JAK2V617F mutation. IPSET score showed high risk of thrombosis and because of comorbidities complete tests of hemostasis were performed. Thromboelastography (TEG) revealed a hypocoagulation state with increased fibrinolytic activity (r=14.6min,k=5.4min,CI=-9.6), moderately decreased synthesis of clotting factors with normal synthesis of coagulation inhibitors; INR 1.35, APTT 41.1s, fibrinogen 2.35, APC-R 292.6s, AT-III 91, proteinC 62%, proteinS 78%. TEG showed normal platelet function, 100% aggregation and 0% inhibition with ADP and arachidonic acid. Coagulation abnormalities were presumably from patient’s liver disease and with the presence of varices the risk of bleeding was considered substantial. Cytoreduction therapy with hydroxyurea 1g/d was started, with aspirin 100 mg/d, but the patient was not given anticoagulation treatment, as chronic PVT was asymptomatic and combined with cirrhosis. One month later the patient had stable Plt 300x10G/l and was referred to gastroenterologist for further treatment.
Conclusions: Chronic PVT complication with cirrhosis in a patient with ET requires detailed investigation of hemostasis and careful, complex treatment approach.
To cite this abstract in AMA style:Petkova N, Hristoskova R, Danchev D, Nikolov I, Raynov J. Essential Thrombocythemia Complicated with Portal Vein Thrombosis and Liver Cirrhosis – A Case Report [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/essential-thrombocythemia-complicated-with-portal-vein-thrombosis-and-liver-cirrhosis-a-case-report/. Accessed November 26, 2020.
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