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Antiphospholipid syndrome as the second cause of non-tumorous Budd-Chiari syndrome

https://doi.org/10.1016/S0168-8278(94)80140-1Get rights and content

In a prospective study of 22 patients with non-tumorous Budd-Chiari syndrome, four were found to have the antiphospholipid syndrome with no other cause of hepatic vein thrombosis. All four patients were young women. The antiphospholipid syndrome was secondary to systemic lupus in one case, to a “lupus-like disease” in another, and apparently primary in the remaining two cases. Two patients died. The other two are in good health on chronic oral anticoagulation. In our experience, the antiphospholipid syndrome is a frequent cause, after myeloproliferative disorders, of non-tumorous Budd-Chiari syndrome. In such patients, long-term anticoagulation may prevent recurrence or extension of thrombosis.

References (38)

  • AverbuchM et al.

    Budd-Chiari syndrome as the major thrombotic complication of systemic lupus erythematosus with the lupus anticoagulant

    Ann Rheum Dis

    (1986)
  • DucobuJ et al.

    Budd-Chiari syndrome in a patient with systemic lupus erythematosus

    Acta Clin Belg

    (1987)
  • SaurezL et al.

    Vascular hepatic disease in childhood associated with lupus anticoagulant factor

    J Clin Nutr Gastroenterol

    (1988)
  • AshersonRA et al.

    Budd-Chiari syndrome, visceral arterial occlusions, recurrent fetal loss and the “lupus anticoagulant” in systemic lupus erythematosus

    J Rheumatol

    (1989)
  • FarrantJM et al.

    Thrombotic cutaneous nodules and hepatic vein thrombosis in the anticardiolipin syndrome

    Clin Exp Dermatol

    (1989)
  • StinsonJ et al.

    Recurrent disseminated intravascular coagulation and fulminant intra-hepatic thrombosis in a patient with the antiphospholipid syndrome

    Am J Hematol

    (1990)
  • AshersonRA et al.

    The hepatic complications of the antiphospholipid antibodies

    Clin Exp Rheum

    (1991)
  • ShapiroS et al.

    Lupus anticoagulant

    Prog Hemost Thromb

    (1982)
  • HarrisEN et al.
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