Management of bleeding in severe factor V deficiency with a factor V inhibitor

Vox Sang. 2014 Jul;107(1):97-9. doi: 10.1111/vox.12134. Epub 2014 Feb 12.

Abstract

Factor V (FV) inhibitor arises rarely after using fresh frozen plasma (FFP) to treat inherited FV deficiency and is often a real therapeutic challenge. Here, we report a patient with a severe FV deficiency who developed such an inhibitor and was then treated with recombinant activated FVII (rFVIIa) and platelet concentrates (PC). Monitoring was assessed by thrombin generation assay (TGA). PC were more effective than rFVIIa in treating bleeding, but there was no correlation between the TGA results and clinical efficacy.

Keywords: factor V inhibitor; fresh frozen plasma; platelet concentrate; recombinant activated factor VII; thrombin generation assay.

Publication types

  • Case Reports

MeSH terms

  • Factor V / antagonists & inhibitors*
  • Factor V Deficiency / complications*
  • Factor V Deficiency / genetics
  • Factor VIIa / pharmacology*
  • Hemoglobins / metabolism
  • Hemorrhage / drug therapy*
  • Hemorrhage / etiology
  • Humans
  • Male
  • Middle Aged
  • Mutation, Missense / genetics
  • Plasma
  • Recombinant Proteins / pharmacology
  • Thrombin / immunology
  • Treatment Outcome

Substances

  • Hemoglobins
  • Recombinant Proteins
  • Factor V
  • recombinant FVIIa
  • Factor VIIa
  • Thrombin