Pharmacokinetics of von Willebrand factor and factor VIII coagulant activity in patients with von Willebrand disease type 3 and type 2

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia Vol. 4; no. S3; pp. 44 - 47
• Main Author: Menache, D
• Format: Journal Article
• Language: English
• Published: Oxford UK Blackwell Science Ltd 01.01.1998
• Subjects: Factor VIII - administration & dosage; Factor VIII - pharmacokinetics; FVIII decay; FVIII synthesis; Humans; Infusions, Intravenous; pharmacokinetics of FVIII; pharmacokinetics of vWF; von Willebrand Diseases - drug therapy; von Willebrand Factor - administration & dosage; von Willebrand Factor - pharmacokinetics; vWF decay
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1046/j.1365-2516.1998.0040s3044.x

Nine patients with von Willebrand disease type 3, six with type 2B, one with type 2A, and one patient with type 1/2N were infused with one dose of ≈50 or 100 IU ristocetin cofactor activity (RCoF) per kg body weight of von Willebrand factor (vWF) (Human), a product with a very low content of factor VIII (FVIII). Blood samples were collected over 96 h. The data for RCoF and vWF antigen (vWF:Ag) were fitted to a 1‐compartment model decay. The data for FVIII:C were fitted to a model with a linear time ‘synthesis’ term and a 1‐compartment decay. Results in von Willebrand disease type 3 patients (nine patients; 10 infusions) indicated a volume of distribution of 39.9 and 39.8 mL kg−1 for RCoF and vWF:Ag, respectively. The FVIII:C rate of synthesis was 6.4 U dL−1 h−1 (range: 4.4–8.8). The decay rates for FVIII:C, RCoF, and vWF:Ag were 0.041 (h−1) [t1/2: 16.9 h]; 0.061 (h−1) [t1/2: 11.3 h] and 0.006 (h−1) [t1/2: 12.4 h], respectively. In patients with von Willebrand disease type 2 (n = 8) the RCoF mean volume of distribution was 46 mL kg−1. The factor VIIIC mean rate of synthesis was 5.5 U dL−1h−1 and the decay rate 0.043 (h−1) [t1/2: 16.1 h]. The rate of decay for RCoF and vWF:Ag were 0.050 (h−1) [t1/2: 13.9 h] and 0.044 (h−1) [t1/2: 15.7 h], respectively.