Abstract
Inherited factor VII (FVII) deficiency is the most frequently observed rare bleeding disorder. The clinical symptoms are extremely variable, and patients may be asymptomatic or may present with life-threatening bleeding disorders. FVII activity (FVII: C) values of 10-15% are considered safe for maintenance of hemostasis, and replacement therapy is recommended for some types of surgical procedures. Twenty-three adult patients with FVII:C deficiency are followed-up in the Ostrava Haemophilia Treatment Centre. Eleven patients underwent a total of fourteen invasive procedures between 2008 and 2013. In terms of replacement therapy, nine patients received plasma-derived FVII and five patients received activated recombinant FVII. None of the patients had excessive blood loss during surgery, and there were no bleeding or other complications during post-operative treatment. In addition, there were no thromboembolic events related to the use of replacement therapy.
Highlights
Inherited coagulation factor VII deficiency is the most frequently observed rare inherited coagulation disorder
Clinical manifestations of factor VII (FVII):C deficiency include predominant bleeding into tissues rich in tissue factor such as the brain, intestines, uterus, placenta, and lungs, and bleeding episode onset may be associated with a surgical procedure [2]
FVII: C values of 10-15% are considered a hemostatic minimum, and replacement of FVII is recommended for surgical procedures
Summary
Inherited coagulation factor VII deficiency is the most frequently observed rare inherited coagulation disorder. Clinical manifestations of FVII:C deficiency include predominant bleeding into tissues rich in tissue factor such as the brain, intestines, uterus, placenta, and lungs, and bleeding episode onset may be associated with a surgical procedure [2]. Considering the safety risks, the replacement of choice is activated recombinant FVII (rFVIIa), or plasma-derived FVII concentrate (pdFVII); fibrin glue may be used in certain circumstances to support local hemostasis (e.g. in stomatosurgery). When these preparations are not available, concentrate of prothrombin complex (PCC) factors or plasma may be used [1]
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