The case of a 65 year old woman referred for further evaluation of back pain and with abnormalities at ultrasound including increase in portal vein diameter and splenomegaly is presented. Other tests, including bone marrow biopsy and Doppler ultrasound, led to a diagnosis of portal vein thrombosis secondary to chronic myeloid leukemia. After prompt cytoreductive therapy with leukapheresis and hydroxyurea, resolution of portal vein thrombosis and portal hypertension was achieved within a in one-month period. An abnormal increase of cells in circulating blood may lead to portal vein thrombosis in patients with myeloproliferative disorders such as chronic myeloid leukemia. Chronic myeloid leukemia is an unusual cause of portal vein thrombosis and portal hypertension. Early administration of cytoreductive therapy may lead to the resolution of portal vein thrombosis. In this report, etiopathogenetic factors of portal vein thrombosis and the role of cytoreductive therapy in the dissolution of thrombosis are discussed.

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