title: Recombinant factor VIIa treatment for asymptomatic factor VII deficient patients going through major surgery.
abstract: Factor VII deficiency is the most common among the rare autosomal recessive coagulation disorders worldwide. In factor VII deficient patients, the severity and clinical manifestations cannot be reliably determined by factor VII levels. Severe bleeding tends to occur in individuals with factor VII activity levels of 2% or less of normal. Patients with 2-10% factor VII vary between asymptomatic to severe life threatening haemorrhages behaviour. Recombinant factor VIIa (rFVIIa) is the most common replacement therapy for congenital factor VII deficiency. However, unlike haemophilia patients for whom treatment protocols are straight forward, in asymptomatic factor VII deficiency patients it is still debatable. In this study, we demonstrate that a single and very low dose of recombinant factor VIIa enabled asymptomatic patients with factor VII deficiency to go through major surgery safely. This suggestion was also supported by thrombin generation, as well as by thromboelastometry.
authors.affiliation: Sheba Medical Center, Institute of Thrombosis and Haemostasis, Tel Hashomer, Israel.
authors.forename: Tami
authors.initials: T
authors.lastname: Livnat
authors.forename: Boris
authors.initials: B
authors.lastname: Shenkman
authors.forename: Galia
authors.initials: G
authors.lastname: Spectre
authors.forename: Ilia
authors.initials: I
authors.lastname: Tamarin
authors.forename: Rima
authors.initials: R
authors.lastname: Dardik
authors.forename: Amnon
authors.initials: A
authors.lastname: Israeli
authors.forename: Avraham
authors.initials: A
authors.lastname: Rivkind
authors.forename: Moshe
authors.initials: M
authors.lastname: Shabtai
authors.forename: Uri
authors.initials: U
authors.lastname: Marinowitz
authors.forename: Ophira
authors.initials: O
authors.lastname: Salomon
mesh_terms: D000368:Aged; D058070:Asymptomatic Diseases; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D004334:Drug Administration Schedule; D017558:Elective Surgical Procedures; D005167:Factor VII / Q000235:genetics*; D005168:Factor VII Deficiency / Q000188:drug therapy* / Q000235:genetics; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011994:Recombinant Proteins / Q000627:therapeutic use; D013916:Thrombelastography; D013917:Thrombin / Q000378:metabolism
chemical_list: D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa; D013917:Thrombin