The Budd-Chiari syndrome is a rare but serious condition defined as hepatic venous outflow obstruction. This article presents a case of acute Budd-Chiari syndrome in a 43-year-old woman with activated protein C resistance while she was being treated with intravenous heparin infusion for previously documented deep venous thrombosis. Activated protein C resistance caused by factor V Leiden mutation has emerged as the most prevalent hereditary cause of venous thromboembolism and should be considered as an etiologic factor in patients with Budd-Chiari syndrome.
Reynolds TBBudd-Chiari syndrome. In: Schiff L , Schiff ER, eds. Diseases of the liver, 7th ed. Philadelphia : JB Lippincott, 1993.
2.
Kage M., Arakawa M., Kojiro M., et al. Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome. Gastroenterology1992;102:2081.
3.
Sherlock S., Dooley J.Diseases of the liver and biliary system, 9th ed. Malden. MA: Blackwell Scientific Publications , 1993.
4.
Dahlbäck B.Inherited thrombophilia: resistance to activated protein C as a pathogenetic factor of venous thromboembolism. Blood1995 ;85:607.
5.
Tabernero MD , Tomas JF, Alherca I., et al. Incidence and clinical characteristics of hereditary disorders associated with venous thrombosis . Am J Hematol1991;36:249.
6.
Svensson PJ, Dahlbäck B.Resistance to activated protein C as a basis for venous thrombosis . N Engl J Med1994;330:517.
7.
Ruiz-Arguelles GJActivated protein C resistance as a cause of thrombophilia. Rev Invest Clin1996;48:223.
8.
Koster T., Rosendaal FR, de Ronde F., et al. Venous thrombosis due to poor response to activated protein C: Leiden thrombophilia study. Lancet1993 ;342;1503.
9.
Sun X., Evatt B., Griffin JHBlood coagulation factor Va abnormality associated with resistance to activated protein C in venous thrombophilia. Blood1994;83:3120.
10.
Bertina RM, Koeleman Bpc, Koster T., et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature1994;369:64.
11.
Denninger MH , Beldjord K., Durand F., et al. Budd-Chiari syndrome and factor V Leiden mutation. Lancet1995;345:525.
12.
Mahmoud Aea , Wilde JT, Elias E.Budd-Chiari syndrome and factor V Leiden mutation . Lancet1995 ;345:526.
13.
Mambrini P. , Mallet D., O'Callaghan T., et al. Budd-Chiari syndrome and activated protein C resistance. J Hepatol1996;24:246.
14.
Fickert P., Ranschak H., Kenner L., et al. Acute Budd-Chiari syndrome with fulminant hepatic failure in a pregnant woman with factor V Leiden mutation. Gastroenterology1996;11:1670.
15.
Blanshard C. , Pasi J., Rolles K., et al. Acute Budd-Chiari syndrome treated by liver transplantation in a woman homozygous for factor V Leiden . Eur J Gastroenterol Hepatol1996;8:925.
16.
Mitchell MC , Boitnott JK, Kaufman S., et al. Budd-Chiari syndrome: etiology, diagnosis, and management. Medicine1982;61: 199.
17.
Schafer AIBleeding and thrombosis in the myeloproliferative disorders. Blood1984;64: 1.
18.
Boughton BJHepatic and portal vein thrombosis. BMJ1991 ;302: 192.
19.
Nachman RL, Silverstein R.Hypercoagulable states. Ann Intern Med1993 ;119:819.
20.
Teofili L., de Stefano V., Leone G., et al. Hematological causes of venous thrombosis in young people: high incidence of myeloproliferative disorders as underlying disease in patients with splanchnic venous thrombosis. Thromb Haemost1992;67(3):297.
21.
Valla D., Casadevall N., Lacombe C., et al. Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. Ann Intern Med1985;103:329.
22.
Valla D., Casadevall N., Huisse MG, et al. Etiology of portal vein thrombosis in adults. A prospective evaluation of primary myeloproliferative disorders. Gastroenterology1988;94:1063.
23.
Holding S.Incidence of acquired prothrombotic defects in the myeloproliferative disorders . Thromb Haemost1993;69:1218.
24.
Holding S., Smith J., et al. Acquired coagulation defects in myeloproliferative disorders . Thromb Haemost1991;65:1018.
25.
Bucalossi A. , Marotta G., Bigazzi C., et al. Reduction of antithrombin III, protein C, protein S levels and activated protein C resistance in polycythemia vera and essential thrombocythemia patients with thrombosis . Am J Hematol1996;52:14.