Myeloproliferative disease in the pathogenesis and survival of Budd-Chiari syndrome.
نویسندگان
چکیده
We studied the etiology, diagnosis and natural course of myeloproliferative disease (MPD) in 40 consecutive patients with Budd-Chiari syndrome (BCS). In 38% of the BCS patients with MPD another etiological factor was found. JAK2 mutation was present in 41% of the tested BCS patients. Survival was not significantly affected by the presence of MPD.
منابع مشابه
[Budd Chiari syndrome].
Primary Budd-Chiari syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assess...
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Budd-Chiari syndrome (BCS) is a rare clinicopathological condition characterized by occlusion of the major hepatic veins with or without membranous obstruction (MOVC)of the inferior vena cava (IVC) at its hepatic portion. Ascites, hepatomegaly and upper abdominal pain constitute the classical triad of symptoms, and the disease usually progresses to congenital liver cirrhosis and portal hyperten...
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متن کاملBudd-Chiari Syndrome in a Patient with JAK-2 Mutation without Myelo- prolifrative Disorder
A 41-year-old woman presented with right upper quadrant abdominal pain, found to have a primary buddchiari syndrome secondary to right hepatic vein thrombosis. Her thrombophilia workup revealed a JAK2 mutation is a setting of no signs of myeloproliferative disorders. JAK2V617F mutation could be a pro-coagulant to thrombosis even without myeloprolifrative disorders.
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ورودعنوان ژورنال:
- Haematologica
دوره 91 12 شماره
صفحات -
تاریخ انتشار 2006