ABSTRACT

Budd-Chiari Syndrome is characterized with hepatic venous obstruction. Myeloproliferative diseases are one of the main causes of Budd-Chiari Syndrome. Although Policitemia Vera is the most concomitant disorder, its association with primer mylefibrosis is not uncommon. In this report, an acut pulmonary thromboembolism, which seen very rare was presented in a woman, 31 years of age, diagnosed with idiopatic myelofibrosis and Budd-Chiari Syndrome and treated by anticoagulant treatment. Primary myelofibrosis cases could have a risk for developing acute pulmoner thromboembolism even they under the anticoagulant treatment. So it is crucially important that these patients should be closely monitored for thromboembolic events even if their continuing anticoagulant treatment