w. Total rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16–31. None of our patients developed excessive bleeding including those in whom FVII:C trough levels returned nearly to the baseline level on the first post-op day. Preliminary results demonstrate that rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients

undergoing orthopaedic surgery. Inherited factor VII (FVII) deficiency has an estimated incidence of 1:300 000–1:500 000 in the general population and an autosomal recessive pattern of inheritance [1, 2]. In Poland, FVII deficiency is http://www.selleckchem.com/products/rxdx-106-cep-40783.html the fourth most common inborn bleeding PF-02341066 purchase diathesis with 195 cases registered in the nationwide database of inherited bleeding disorders [3]. Haemorrhagic manifestations in the affected individuals are variable and correlate poorly with plasma FVII activity levels (FVII:C) [4, 5]. In severely affected cases, however, significant bleeding problems have been observed including spontaneous haemarthroses

resulting in advanced arthropathy. In such cases orthopaedic surgery may be required. For bleeding prevention in FVII-deficient patients undergoing surgery therapeutic options comprise various FVII-containing preparations such as fresh frozen plasma (FFP), prothrombin complex concentrates (PCC), plasma derived FVII (pdFVII) concentrates and recombinant-activated FVII (rFVIIa) [6]. Since patients undergoing surgery may require prolonged administration of FVII-containing preparation, FFP is currently not recommended due to fluid overload. Moreover, FFP carries the risk of blood borne virus transmission. PCC concentrates contain factor II, MCE factor IX, factor X and highly variable amounts of FVII and are considered to have significant thrombogenic potential; they are therefore not recommended for patients requiring frequent (e.g. daily) infusions of FVII. Plasma-derived FVII concentrates subjected to virus inactivation have been proved effective in the management of FVII

deficient patients undergoing a variety of surgical procedures [7, 8]. Theoretically, however, plasma-derived products, are still associated with some risk of transfusion-transmitted infections. The treatment of choice for FVII-deficient patients therefore seems to be rFVIIa, which provides the missing protein in a low-volume preparation, and is devoid of other human or animal proteins [9]. Despite the broad use of rFVIIa in FVII deficiency, the published data on dosage and treatment schedules in surgical setting are scarce [6, 10]. The aim of our article is to present the preliminary results of the novel treatment regimen for haemostatic management of FVII deficient patients undergoing orthopaedic surgery (Table 1). The study comprised five successive patients, four women and one man, aged 20–78 years, with inherited FVII deficiency (FVII:C below 10 IU dL−1) who required joint surgery.