Recommendation for prophilaxy in congenital factor VII deficiency (comparison between factor VII deficiency and classic hemophilia)

Background: Inherited factor VII (FVII) deficiency is the most common among rare congenital bleeding disorders, accounting for one symptomatic individual per 500000 populations, and is characterized by autosomal recessive inheritance. Disorder is usually detected only in the homozygous state. Aims: Bleeding in patients affected by inherited factor VII deficiency is extremely heterogeneous concerning sites and severity. Hemorrhagic predisposition in affected patients correlates poorly with plasma factor VII activity levels. Individuals with this deficiency may have spontaneous intracranial hemorrhage and frequent mococutaneous bleeding. Other clinical manifestations are hemarthrosis and crippling arthropathy (comparable to patients with classic hemophilia), haematuria,menorrhgia. Factor VII is vitamin K-dependent factor, but vitamin K administration is of no therapeutic benefit in the treatment of hereditary deficiencies. The design protocol of on demand and prophylactic in these patients is very important. Methods: I have 110 patients of hemophilia and rare bleeding disorders in my centre. 6 of them (2 lives, 4 death) have congenital factor VII deficiency and 67 of them have hemophilia A. I compare times of bleeding, severity of them and risks of bleeding in this two groups. Findings on descriptive statistics (contain mean and sum and frequency) were analyzed. Results: The risk of bleeding, especially brain hemorrhage, in congenital factor VII deficiency is greater than classic hemophilia. Conclusions: Although prophylactic management of congenital factor VII deficiency is not recommended in text books, but considering the risks of disease this management with long-acting Novoseven is recommended