Article Text

Download PDFPDF
Factor V Leiden related Budd-Chiari syndrome
  1. P Deltenrea,
  2. M-H Denningerb,
  3. S Hillairea,
  4. M-C Guillinb,
  5. N Casadevalld,
  6. J Brièrec,
  7. S Erlingera,
  8. D-C Vallaa
  1. aService d'Hépatologie, Hôpital Beaujon, Clichy, France, bLaboratoire d'Immunohématologie, Hôpital Beaujon, Clichy, France, cService d'Hématologie Clinique, Hôpital Beaujon, Clichy, France, dLaboratoire d'Hématologie, Hôpital Raymond Poincaré, Garches, France
  1. Dr D-C Valla, Service d'Hépatologie, Hôpital Beaujon, 100, Boulevard du Général Leclerc, 92118 Clichy, France.dominique.valla{at}


BACKGROUND The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described.

AIMS To assess the specific features of factor V Leiden related Budd-Chiari syndrome.

PATIENTS Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis.

METHODS Standardised chart review.

RESULTS Factor V Leiden was found in 20 patients (31% (95% CI 20–43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50–90) v 14% (95% CI 3–24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19–61)v 7% (95% CI 0–14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava.

CONCLUSIONS In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis—particularly of the inferior vena cava—or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy.

  • thrombophilia
  • Budd-Chiari syndrome
  • inferior vena cava obstruction
  • myeloproliferative disorders
  • ischaemic necrosis
  • Abbreviations used in this paper

    alanine aminotransferase
  • Statistics from

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.