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Buddi-Chiari syndrome associated with hypereosinophilic syndrome: A case report
Zhaoxia Li1, Nan Li1, Zhuhui Ji1
1Department of Hepatology, The First Hospital of Jilin University, Jilin Province, China.
Insights
This case report details a rare instance of Budd-Chiari Syndrome (BCS) co-occurring with hypereosinophilic syndrome in a 33-year-old female. The study suggests a potential association between these conditions, highlighting the need for further research.
Area of Science:
- Hepatology
- Hematology
- Rare Diseases
Background:
- Budd-Chiari Syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction, with varying etiologies globally.
- Hepatic venous obstruction is common in Western countries, often linked to hypercoagulable states.
- Inferior vena cava obstruction is more prevalent in Asia, with slower progression and less epidemiological data.
Observation:
- A 33-year-old female presented with bleeding symptoms and elevated eosinophils.
- Abdominal ultrasound revealed hepatosplenomegaly and suspected arteriovenous fistulas.
- Diagnostic workup confirmed Budd-Chiari Syndrome co-existing with hypereosinophilic syndrome and a JAK2V617F mutation.
Findings:
- The patient underwent successful endovascular stent implantation and anticoagulation therapy.
- Follow-up CT scans showed significant reduction in hepatic abnormalities and patent stents.
- This case highlights a rare association between Budd-Chiari Syndrome and hypereosinophilic syndrome.
Implications:
- This case report suggests a potential link between Budd-Chiari Syndrome and hypereosinophilic syndrome.
- Further large-scale studies are needed to elucidate the etiology and pathogenesis of this combined condition.
- Identifying this association may offer new therapeutic targets and strategies for BCS management.
Rationale:
Budd-Chiari Syndrome (BCS) is a relatively rare clinical disorder with a wide range of symptoms, caused by the obstruction of the hepatic venous outflow. The etiology and pathogenesis of BCS vary in different countries and regions. In Western countries, hepatic venous obstruction is the most common type, and its main cause is closely related to the hypercoagulable state of the body. Inferior vena cava obstruction is common in Asia, and its etiology progresses slowly due to the lack of epidemiological data. [3] Here, we report a rare case of BCS associated with the hypereosinophilic syndrome and discuss the possible causal relationship between the two.
Patient Concerns:
The patient was a 33-year-old female with intermittent epistaxis, gum bleeding, and excessive menstrual flow for the past 6 months. The routine blood tests showed elevated levels of eosinophils, and the liver function test showed mildly elevated levels of γ-glutamyl transpeptidase and alkaline phosphatase, and abdominal ultrasound showed hepatosplenomegaly and suspicion of intrahepatic arteriovenous or arteriovenous-portal fistula.
Diagnoses:
Finally, through the improvement of bone marrow aspiration, digital subtraction angiography and gene detection, the diagnosis of BCS combined with hypereosinophilic syndrome was confirmed, and JAK2V617F mutation was highly associated with it.
Interventions:
The patient received endovascular stent implantation and regular oral rivaroxaban anticoagulation therapy after operation.
Outcomes:
Seven months later, enhanced computed tomography (CT) of the hepatobiliary showed that the hepatic bruise-like changes were significantly reduced compared with before, and the right hepatic vein and the right perihepatic vein stent were left in place with a good filling of contrast in the stent.
Lessons:
The patient, in this case, was finally diagnosed with BCS combined with hypereosinophilic syndrome, and to our knowledge, such case reports are rare. Our case report suggest an association between BCS and hypereosinophilic syndrome, but relevant studies are minimal, we hope to conduct larger and higher quality studies on these patients in the future, to provide new directions and basis for the etiology and pathogenesis of these diseases, as well as provide new targets and ideas for clinical treatment.
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