001); clearance significantly decreased with increased VWF:Ag (P = 0.002). Annualized bleeding rate in patients treated with 3× per week rFVIII-FS significantly correlated with VWF:Ag and age (P = 0.038 and 0.021 respectively). PK parameters as well as the clinical outcome significantly correlated with endogenous VWF:Ag. The improved clinical outcome in subjects with high

VWF:Ag levels may be explained by VWF:Ag influence on FVIII PK. “
“Increase of factor VIII activity (FVIII) after physical exercise has been reported in healthy subjects and small-scale studies in patients with coagulopathies. The aim was to study whether moderate and mild haemophilia A patients are able to increase their endogenous FVIII activity levels by physical activity. We studied changes in FVIII activity levels after high-intensity exercise in 15 haemophilia A patients, 20–39 years, eight with moderate, GSK1120212 seven with mild haemophilia. Patients cycled until volitional learn more exhaustion, blood samples were drawn before and 10 min after the exercise test. FVIII activity increased 2.5 times (range 1.8–7.0 times), for both severities. Absolute increases were markedly different: median 7 IU dL−1 (range 3–9 IU dL−1) in patients with moderate, compared to 15 IU dL−1 (range 6–62 IU dL−1) in mild haemophilia patients. VWF and VWFpp increased independently

of severity; median 50% (range 8–123%) and median 165% (range 48–350%), respectively, reflecting acute release of VWF. These observations may be used to promote high-intensity activities

before participating in sports for moderate and mild haemophilia A patients, to reduce bleeding risk. Further studies are warranted to fully appreciate the clinical significance of exercise on different levels of intensity in patients with mild and moderate haemophilia A. “
“Summary.  Recombinant factor VIIa is indicated MCE for treatment of bleeding episodes in patients with haemophilia A or B with inhibitors; in FVII deficiency and in Glanzmann’s thrombasthenia. The aim of the study reported here was to compare the pharmacokinetic profiles between two formulations of rFVIIa that are produced in two different cell lines and media: Chinese hamster ovary cells cultured in a serum-free medium (CHO-rFVIIa) and baby hamster kidney cells cultured in a non-human serum-based medium (BHK-rFVIIa). Two clinical trials were performed; one in healthy subjects and the other in patients with congenital haemophilia A or B, with or without inhibitors. Subjects were recruited into a two-way crossover trial and were randomized to receive a dose of CHO-rFVIIa and BHK-rFVIIa. Healthy subjects received one dose of 90 μg CHO-rFVIIa kg−1 bodyweight (bw) in the newly developed room-temperature stable rFVIIa formulation and one dose of 90 μg BHK-rFVIIa kg−1 bw, in the original rFVIIa formulation. Patients with haemophilia received one dose of 270 μg CHO-rFVIIa kg−1 and one dose of 270 μg BHK-rFVIIa kg−1, both in the room-temperature stable formulation.