Coagulation Factor VII Deficiency: A Review of the Clinical Implications and Approaches to Replacement Therapy and Prophylaxis.

Factor VII (FVII) deficiency is a rare bleeding disorder that can present as either inherited or acquired. Inherited FVII deficiency arise from mutations in the F7 gene, resulting in a highly heterogeneous and wide range of phenotypic expressions from asymptomatic individuals to those experiencing severe and life-threatening hemorrhagic episodes. Notably, the severity of symptoms is typically not correlated with plasma FVII levels. Diagnosis generally involves a combination of coagulation assays, including prothrombin time (PT), assessment of FVII activity and antigenic levels, and a thorough consideration of the patient's clinical context. Management of FVII deficiency requires personalized treatment strategies. For inherited forms, on-demand replacement therapy is commonly employed, utilizing recombinant activated FVII, plasma-derived FVII, prothrombin complex concentrates, or fresh frozen plasma during bleeding episodes, as well as for prophylactic measures. Long-term prophylaxis is particularly critical for patients with severe deficiencies who have a history of central nervous system or gastrointestinal bleeding, especially those at an elevated risk of subsequent major or potentially life-threatening bleeding events. This review evaluates current therapeutic approaches, highlights emerging challenges in the management of FVII deficiency, and underscores the importance of tailored treatment strategies aimed at optimizing patient outcomes.