Budd-Chiari syndrome caused by hepatic vein thrombosis in a patient with myeloproliferative disorder.
نویسندگان
چکیده
We report a 24-year-old Japanese female hospitalized with jaundice and ascites. She exhibited hepatosplenomegaly, severe liver dysfunction, and slight polycythemia with an increase in serum levels of beta-thromboglobin and platelet factor 4. Bone marrow was hypercellular with an increase in progenitor cells. The aggregation response of platelets to ADP and to collagen was markedly increased. Venography revealed narrowed hepatic veins with "spider web' sign. Liver biopsy revealed hepatic congestion. Budd-Chiari syndrome was diagnosed, and was thought to be due to thrombosis related to myeloproliferative disorder. Liver transplant was successful in relieving symptoms.
منابع مشابه
Budd-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:
- Internal medicine
دوره 35 11 شماره
صفحات -
تاریخ انتشار 1996