Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia

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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Journal
Cureus
Published
2025-03-17
DOI
https://doi.org/10.7759/cureus.80700
Primary Topic
Myeloproliferative Neoplasms: Diagnosis and Treatment
Type
article
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article

Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia

Leonor Gama, Rodrigo Morgado, Monique de Oliveira e Silva, Ana Santos e Silva et al.
Cureus
Myeloproliferative Neoplasms: Diagnosis and Treatment
article

Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia

Leonor Gama, Rodrigo Morgado, Monique de Oliveira e Silva, Ana Santos e Silva, Josiana Duarte
article en

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.

CureusVol. 17(3)
Administração Regional de Saúde de Lisboa e Vale do Tejo (PT)
Zero hunger
Openalex Percentile: Top 93%
Myeloproliferative Neoplasms: Diagnosis and Treatment
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Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia — Leonor Gama, Rodrigo Morgado, et al. · Cureus (2025) | TGRS Research Map | TGRS