Congenital Bleeding Disorders pp 239-259 | Cite as
Congenital Factor VII Deficiency
Abstract
Congenital factor VII (FVII) deficiency with a prevalence of 1 per 500,000 is the most common rare bleeding disorder. Although the majority of patients have mild bleeding tendency, a considerable number of them present life-threatening bleedings such as central nervous system (CNS) and gastrointestinal (GI) bleedings. Although the diagnosis of congenital FVII deficiency is straightforward with isolated prolonged prothrombin time (PT) and decreased FVII level, differential diagnosis of acquired FVII deficiency should be considered. Several therapeutic options such as fresh frozen plasma (FFP), plasma-derived FVII (pd-FVII), prothrombin complex concentrate (PCC), activated PCC (aPCC), and, more recently, recombinant activated FVII (rFVIIa) are available for treatment of patients with FVII deficiency. New therapeutic options significantly improved the quality of life in patients with congenital FVII deficiency.
Keyword
Factor VII deficiency Rare bleeding disorder Clinical manifestations Diagnosis TreatmentReferences
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