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• W2531326162 abstract "To the Editor: A defect in the anticoagulant response to activated protein C (APC) is a recently identified hereditary mechanism of thrombophilia (1) linked to a single point mutation in the factor V gene (2). We report a case of Budd-Chiari syndrome associated with APC resistance and a myeloproliferative disorder. A 21-year-old man was admitted in February 1995 with a 2-month history of abdominal pain, weakness, and swelling. Physical examination showed hepatosplenomegaly, jaundice, ascites, and peripheral oedema. Total bilirubin was 40 pmolll, aspartate aminotransferase 80 U/I gammaglutamyl transpeptidase 241 U/I, alkaline phosphatases 257 U/l, and prothrombin ratio 55%, with factor V 43%. Hepatic Doppler ultrasonography, magnetic resonance imaging, and liver histology demonstrated Budd-Chiari syndrome, with occluded hepatic veins. Red cells were 6.7~10’*/1, haemoglobin 16.5 gidl, packed cell volume 54%, white cells 10x 109/1, and platelets 438x 109/1. Red cell mass was increased to 40 ml/kg, vitamin B12 to 1550 rig/l and leukocyte alkaline phosphatase score to 65. Serum erythropoietin was 5.9 mUI/ml (normal 4-16). Bone-marrow biopsy was hypercellular with an increased number and polymorphism of megakaryocytes. There was no myelofibrosis. These results were consistent with myeloproliferative disease. Thrombophilia screening showed 61% protein C, 100% protein S, 95% antithrombin III, and negative lupus anticoagulant. The APC resistance test ratio was uninformative because of increased activated partial thromboplastin time. Polymerase chain reaction genomic DNA analysis (2) indicated heterozygosity for factor V 4506 mutation. Although there was no familial history of thrombosis, the patient’s father presented APC resistance, as determined by the APC-dependent prolongation of the activated partial thromboplastin time, and he was also heterozygous for the factor V 4506 mutation. The patient was discharged on a low-sodium diet, with venesection, and chemotherapy with pipobroman. One month later, the prothrombin ratio reached 6.5%, and the patient was treated with vitamin K antagonists. Although myeloproliferative disorders are the most common aetiology of Budd-Chiari syndrome, deficiencies in antithrombin III, protein C, protein S, and lupus anticoagulant have also been incriminated. APC resistance seems to be the most prevalent cause of thrombophilia, since it is detected in 2&30% of patients with unexplained thromboembolism, compared with 2-5% of healthy individuals (1). Recently, two cases of Budd-Chiari syndrome were reported, in which APC resistance was associated with protein S deficiency (3) or myeloproliferative disease (4). However, one case of portal thrombosis associated with APC resistance and with no other aetiology, suggests that APC resistance can be implicated in this pathology (5). Our case underlines the possibility of multifactorial aetiology in Budd-Chiari syndrome and suggests that screening for APC resistance may be justified in all patients with Budd-Chiari syndrome or portal thrombosis, even if another predisposing factor has already been identified. For patients with APC resistance, long-term anticoagulant therapy and familial screening for APC resistance may be warranted." @default.
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• W2531326162 date "1996-01-01" @default.
• W2531326162 modified "2023-09-27" @default.
• W2531326162 title "Budd-Chiari syndrome aud activated protein C resistance" @default.
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