Factor V, constitutional deficiency in

(Owren disease, parahemophilia)

Very rare, hemorrhagic disease. Autosomal recessive transmission  of a mutation in the F5 gene (1q24.2) causing congenital coagulation factor V (proaccelerine) deficiency. Factor V plays an essential role in the conversion of prothrombin into thrombin by factor Xa. The formation of fibrin is thus delayed. Only homozygous patients are symptomatic: bruising, epistaxis, post-traumatic bleeding. Deep hematomas and hemarthroses are rare.

The factor V circulating level is less than 10 % in homozygotes (between 30 and 50 % in heterozygotes): a factor V higher than 25 % is enough for normal hemostasis, which can be obtained by a transfusion of FFP (fresh frozen plasma).


Anesthetic implications: 

repeated transfusions of FFP to maintain a blood levels of factor V greater than 15-25 %, the efficacy of which can be monitored by thromboelastography.

Not to be confused with the resistance of factor V (of Leiden) (see this term) to protein C, which concerns the same protein but causes thrombophilia.


References : 

-         El Koraïchi A, Mokhtari M, Ghannam M , Mekaouri N, El Haddoury M, Ech-Cherif El Kettani S.
Déficit en facteur V et circoncision : gestion périopératoire. A propos d’un cas clinique. 
Ann Fr Anesth Réanim 2011 ; 30 : 377-8.

-         Carpenter SL, Abshire TC, Anderst JD,et al. 
Evaluating for suspected child abuse : conditions that predispose to bleeding. 
Pediatrics 2013; 131: e1357-73.

-         Bonhomme F, Schved J-F, Giansily-Blaizot M, Samama C-M, de Moerloose P. 
Déficits rares de la coagulation et gestes invasifs. 
Ann Fr Anesth Réanim 2013 ; 32 : 198-205. 

-        Derwall M, Grottke O.
Coagulation management for a Caesarian delivery in a mother with severe homozygous Factor V deficiency.
J Clin Anesth 2021 ; 74 : 110402


Updated: October 2021