Parahemophilia: new insights into factor v deficiency

Semin Thromb Hemost. 2013 Sep;39(6):607-12. doi: 10.1055/s-0033-1349224. Epub 2013 Jul 26.

Abstract

Blood coagulation factor V (FV) plays a pivotal role in blood coagulation. It is found in both plasma and in platelets and has a profound impact on thrombin generation. Deficiency of this clotting factor due to inherited or acquired conditions results in a broad spectrum of bleeding symptoms. Surprisingly however, some patients with undetectable levels of FV experience relatively mild bleeding. The aim of this review is to highlight this rare coagulation factor disorder and touch upon its clinical manifestations, diagnosis, and treatment. Furthermore, recent advances that shed new light on the importance of platelet FV and other modifiers which influence bleeding tendencies in severe FV deficiency will be discussed.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Blood Platelets / metabolism*
  • Factor V / genetics
  • Factor V / metabolism*
  • Factor V Deficiency / blood*
  • Factor V Deficiency / genetics
  • Factor V Deficiency / therapy
  • Genetic Predisposition to Disease
  • Hemorrhage / blood*
  • Hemorrhage / genetics
  • Hemorrhage / prevention & control
  • Humans
  • Mutation

Substances

  • Factor V