Extract
Factor-VII deficiency is a rare autosomal recessive disorder
affecting approximately one in 500,000
persons1. The risk
of bleeding complications depends on the factor-VII level, and patients with
factor-VII activities of <1% are at risk for spontaneous epistaxis,
menorrhagia, bleeding in the oral cavity, hemarthroses, and postoperative
bleeding; patients with activities ranging from 1% to 5% are moderately
affected; and those with activities of >5% are mildly
affected2. As a
result of its short half-life of six hours, factor-VII-replacement strategies
require frequent infusions of factor preparations. Historically, options for
treatment of or prophylaxis against bleeding in factor-VII-deficient patients
included use of fresh-frozen plasma and plasma-derived concentrates of vitamin
K-dependent coagulation factors (prothrombin complex concentrates). Potential
complications associated with these products include infection with
blood-borne pathogens as well as volume overload with fresh-frozen plasma and
thrombotic events with prothrombin complex concentrates.