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Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia
Ana Santos E Silva1, Rodrigo Morgado2, Monica Ferro Silva1
1Internal Medicine, Unidade Local de Saúde do Litoral Alentejano, Santiago do Cacém, PRT.
Abstract:
Budd-Chiari syndrome (BCS) is a clinical condition defined by the obstruction of hepatic venous outflow, resulting in portal hypertension and subsequent hepatic dysfunction. The syndrome is frequently associated with prothrombotic states, among which myeloproliferative neoplasms represent the most prevalent underlying etiology. The clinical presentation of BCS is highly variable, ranging from a mild and insidious disease course to an acute condition associated with liver failure. This heterogeneity in presentation often leads to delays in diagnosis. This report describes a rare case of BCS as the first manifestation of essential thrombocythemia (ET) in a young male patient, emphasizing the importance of early diagnosis and multidisciplinary management for better outcomes.
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