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

D Menache
Haemophilia: the Official Journal of the World Federation of Hemophilia 1998, 4: 44-7
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 approximately 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-1 h-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.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Trending Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"