Korean J Gastroenterol.  2002 Jan;39(1):64-67.

A Case of Budd-Chiari Syndrome with Essential Thrombocythemia

• Seo BG
• Han SY
• Lee JH
• Jang JS
• Lee HS
• Roh MH
• Choi SR
• Shin WW

Abstract

Budd-Chiari syndrome is an acute or subacute obstruction of hepatic venous outflow which causes post-sinusoidal portal hypertension, esophageal varix, and cirrhotic course. Its prognosis is usually poor. The cause and pathogenesis are unclear, but the primary cause is generally thought to be congenital. The main secondary causes are chronic myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, presence of lupus anticoagulant, protein C or protein S deficiency, hypercoagulability due to oral pills, tumor, trauma, and infection. It can be treated by shunt operation, balloon dilatation, stent insertion, or transjugular intrahepatic portosystemic shunt. We report one case of Budd-Chiari syndrome with essential thrombocythemia which has never been reported in Korea.

Keyword

Budd-Chiari syndrome; Essential thrombocythemia

MeSH Terms

Budd-Chiari Syndrome*
Dilatation
Esophageal and Gastric Varices
Hemoglobinuria, Paroxysmal
Hypertension, Portal
Korea
Lupus Coagulation Inhibitor
Myeloproliferative Disorders
Portasystemic Shunt, Surgical
Prognosis
Protein C
Protein S Deficiency
Stents
Thrombocythemia, Essential*
Thrombophilia
Lupus Coagulation Inhibitor
Protein C