title: Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation.
abstract: A 22-year-old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd-Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2-V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post-transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.
authors.affiliation: Division of Gastroenterology and Hepatology             Division of Digestive and General Surgery             Division of Hematology, Niigata University Graduate School of Medical and Dental Sciences             Department of Clinical Laboratory, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Iwasaki
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kawai
authors.forename: Koushi
authors.initials: K
authors.lastname: Oseki
authors.forename: Tadayuki
authors.initials: T
authors.lastname: Togashi
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Shioji
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yamamoto
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Sato
authors.forename: Kenji
authors.initials: K
authors.lastname: Suzuki
authors.forename: Ken
authors.initials: K
authors.lastname: Toba
authors.forename: Minoru
authors.initials: M
authors.lastname: Nomoto
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Hatakeyama
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Aoyagi