Rare coagulation disorders: fibrinogen, factor VII and factor XIII.

P de Moerloose, J-F Schved, D Nugent

Journal: Haemophilia : the official journal of the World Federation of Hemophilia 2017;22 Suppl 5():61-5

PMID: 27405678

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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.

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

DOI: 10.1111/hae.12965

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MeSH Terms: Afibrinogenemia, Blood Coagulation Disorders, Inherited, Factor VII, Factor VII Deficiency, Factor XIII, Factor XIII Deficiency, Fibrinogen, Humans, Mutation, Missense, Registries