血液疾患と輸血ジャーナル

概要

Management of Surgical Procedures in Patients with Inherited F VII Deficiency: Six Years of Experience

Radomira Hrdlickova, Sarka Blahutova, Roman Hajek and Zuzana Cermakova

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.