Address for correspondence:
Yuji Suzuki, MD, PhD
Division of Hepatology, Department of Internal Medicine, Iwate Medical
University School of Medicine
1-1-1 Idaidori, Yahaba-cho, Shiwa-gun, Iwate 028-3694, Japan
Tel: +81-19-651-5111; Fax: +81-19-907-7166
E-mail:yusuzuki@iwate-med.ac.jp
Funding: The authors received no financial support for the
research, authorship, and/or publication of this article.
Conflict of Interest: The authors declared no potential
conflict of interest with respect to the research, authorship, and/or
publication of this article.
Patient Consent: Informed consent was obtained from the patient
to publish his case and medical images.
The total number of figures: 2
Acknowledgments: None
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A 46-year-old man complaining of fatigue and right upper quadrant pain
that lasted for 10 days was referred to our hospital. Physical
examination revealed abdominal distention. Contrast-enhanced computed
tomography revealed complete thrombotic occlusion of the main trunk of
the portal and splenic veins (Figure 1A, arrows), as well as the three
main hepatic veins (Figure 1B, arrows). Laboratory findings revealed
leukocytosis (leukocytes, 25,610/µL) and acute liver failure (alanine
aminotransferase, 3,188 IU/L; aspartate transaminase, 3,804 IU/L; total
bilirubin, 2.4 mg/dL; ammonia, 140 µg/dL; and international normalized
ratio, 2.24). Although low-molecular-weight heparin was administered
subcutaneously, the patient developed grade III hepatic encephalopathy 5
days after admission. Acute liver failure precipitated by acute
Budd–Chiari syndrome and complete portal vein thrombosis was
diagnosed.1 Ten days after admission, emergency
orthotopic liver transplantation was performed. Microscopic examination
of the explanted liver showed massive hepatocyte loss in the
centrilobular region (Figure 2, hematoxylin and eosin staining, 10×).
The patient was eventually diagnosed with JAK2 V617F mutation-positive
myeloproliferative neoplasm and was treated with hydroxyurea and
aspirin, following liver transplantation.2 Over the
5-year follow-up period, he remains well without post-transplant
complications.
Myeloproliferative neoplasms, including JAK2 V617F mutations, are the
most common cause of Budd–Chiari syndrome and non-malignant,
non-cirrhotic portal vein thrombosis.3 We demonstrated
that acute Budd–Chiari syndrome and complete portal vein thrombosis
might occur simultaneously by myeloproliferative neoplasm, resulting in
acute liver failure. Liver transplantation could be effective even in
such a catastrophic condition.