Rare coagulation disorders: fibrinogen, factor VII and factor XIII

P de Moerloose; J-F Schved; D Nugent

doi:10.1111/hae.12965

Rare coagulation disorders: fibrinogen, factor VII and factor XIII

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

Authors

P de Moerloose¹, J-F Schved², D Nugent³

Affiliations

¹ Division of Angiology and Haemostasis, University Hospitals and Faculty of Medicine, Geneva, Switzerland.
² Hemophilia treatment Center, Montpellier, France.
³ Department of Hematology, Children's Hospital of Orange County, Orange, CA, USA.

PMID: 27405678
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