# Budd-Chiari Syndrome as an Initial Presentation of Essential Thrombocythemia

**Authors:** Ana Santos e Silva, Rodrigo Morgado, Monica Ferro Silva, Leonor Gama, Josiana Duarte

PMC · DOI: 10.7759/cureus.80700 · Cureus · 2025-03-17

## TL;DR

A rare case shows that Budd-Chiari Syndrome can be the first sign of essential thrombocythemia, highlighting the need for early diagnosis.

## Contribution

This paper presents a rare clinical case linking Budd-Chiari Syndrome to essential thrombocythemia as its initial manifestation.

## Key findings

- Budd-Chiari Syndrome can be the first sign of essential thrombocythemia in young patients.
- Early diagnosis and multidisciplinary management improve outcomes in such cases.

## 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.

## Linked entities

- **Diseases:** Budd-Chiari syndrome (MONDO:0010947), portal hypertension (MONDO:0005080), liver failure (MONDO:0100192), essential thrombocythemia (MONDO:0005029)

## Full-text entities

- **Diseases:** portal hypertension (MESH:D006975), BCS (MESH:D006502), ET (MESH:D013920), liver failure (MESH:D017093), myeloproliferative neoplasms (MESH:D009369), hepatic dysfunction (MESH:D008107)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC12001152/full.md

## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12001152/full.md

## References

15 references — full list in the complete paper: https://tomesphere.com/paper/PMC12001152/full.md

---
Source: https://tomesphere.com/paper/PMC12001152