Very low activated factor VII and reduced factor VII antigen in familial abetalipoproteinaemia

Thromb Haemost. 1998 Aug;80(2):233-8.

Abstract

Abetalipoproteinaemia is a rare disorder of apolipoprotein B metabolism associated with extremely low plasma concentrations of triglyceride. To discover whether the general positive association between factor VII and triglyceride levels extends to this condition, 5 patients were compared with 18 controls. All patients had a triglyceride below 100 micromol/l. Plasma unesterified fatty acid concentration was normal. Although factor IX activity was only slightly reduced (mean 88% standard) and factor IX antigen was normal, mean activated factor VII in patients was strikingly reduced to 34% of that in controls, a level similar to that found in haemophilia B. The patients' mean factor VII activity and factor VII antigen were also significantly reduced to 54% and 63% of those in controls, respectively. Mean factor XI activity and tissue factor pathway inhibitor activity were reduced in patients to 70% and 75% of control values respectively, while factor XII, factor XI antigen, factor X, prothrombin and protein C were normal.

MeSH terms

  • Abetalipoproteinemia / blood
  • Abetalipoproteinemia / genetics
  • Abetalipoproteinemia / physiopathology*
  • Adult
  • Antigens / blood*
  • Blood Coagulation Factors / metabolism
  • Case-Control Studies
  • Factor VII / immunology*
  • Factor VIIa / metabolism*
  • Fatty Acids, Nonesterified / blood
  • Female
  • Humans
  • Male

Substances

  • Antigens
  • Blood Coagulation Factors
  • Fatty Acids, Nonesterified
  • Factor VII
  • Factor VIIa