Budd-Chiari syndrome associated with hyperthyroidism

We present a case of Budd-Chiari syndrome associated with hyperthyroidism in a 39 year old woman, in whom an urgent liver transplant was necessary, because of subacute evolution with deterioration of clinical state - portocaval encephalopathy- and worsening of liver function tests. A detailed laboratory and radiological study excluded causes of BCS like mycloproliferative disease, infection, tumours, lupus anticoagulant syndrome. Although the patient had low levels of AT III and C Protein caused by liver failure, all members of her family had normal values and hereditary thrombotic status was excluded. Our case was diagnosed based on clinical and laboratory signs of progressive liver failure, radiological imaging revealing hypertrophy of caudate lobe of the liver, occlusion of suprahepatic veins and compression of hepatic portion of the inferior vena cava. This was reduced to a slit and the hemodynamic data reflected a sinusoidal liver block. Anatomo-pathological aspects showed many areas of hemorrhagic necrosis intermingled with small areas of normal liver without cirrhosis. After transplantation the examination of the liver showed a complete occlusion of the right suprahepatic vein by old and recent thrombus and an almost complete occlusion of left suprahepatic vein by a thrombus. No infectious complications or transplant rejection phenomena were detected after transplantation. The patient is doing well 11 months after transplantation, on immunossupression with cyclosporine, corticosteroids and anticoagulation with high doses of coumarinics. We have not found references of association of BCS with hyperthyroidism. Our patient was referred to us with a clinical picture of hyperthyroidism associated with an abnormal prothrombine time. She was treated with propylthiouracil and propranolol but later it was necessary to begin levothyroxine due to secondary hypothyroidism. Just before transplantation a high blood level of factor VIII was found (258% n<150%). Values above normal persisted during and after transplantation (\gr150%). Some cases of hyperthyroidism are accompanied by thrombotic episodes and elevated factor VIII blood levels. Nevertheless the values usually return to normal after correction of hyperthyroidism, which was not the case in our patient. Four members of her family also had high levels of factor VIII (mother, two sisters and daughter presenting respectively FVIIIc: 210%, 220%, 173%, 208% (n<150%). Hyperthiroidism could be a co-factor for thrombotic episodes in a family with high levels of FVIII. Only female members of the family presented high levels of FVIII in a total of 6 females and 3 males studied which could be explained by heredity (linked to cromosome X) or by hormonal influences.