Rare coagulation disorders: fibrinogen, factor VII and factor XIII

Haemophilia. 2016 Jul:22 Suppl 5:61-5. doi: 10.1111/hae.12965.

Abstract

Rare coagulation disorders (RCDs) include the inherited deficiencies of fibrinogen, factor (F) II, FV, combined FV and VIII, FVII, FX, combined FVII and X, FXI, FXIII and combined congenital deficiency of vitamin K-dependent factors (VKCFDs). Despite their rarity, a deep comprehension of all these disorders is essential to really understand haemostasis. Indeed, even if they share some common features each RCD has some particularity which makes it unique. In this review, we focus on three disorders: fibrinogen, FVII and FXIII.

Keywords: bleeding; factor VII; factor XIII; fibrinogen.

Publication types

  • Review

MeSH terms

  • Afibrinogenemia / diagnosis*
  • Afibrinogenemia / drug therapy
  • Blood Coagulation Disorders, Inherited / diagnosis*
  • Blood Coagulation Disorders, Inherited / drug therapy
  • Factor VII / therapeutic use
  • Factor VII Deficiency / diagnosis*
  • Factor VII Deficiency / drug therapy
  • Factor XIII / genetics
  • Factor XIII / therapeutic use
  • Factor XIII Deficiency / diagnosis*
  • Factor XIII Deficiency / drug therapy
  • Fibrinogen / therapeutic use
  • Humans
  • Mutation, Missense
  • Registries

Substances

  • Factor VII
  • Fibrinogen
  • Factor XIII