Von Willebrand Factor (human)
Von Willebrand Factor (human) Uses, Dosage, Side Effects, Food Interaction and all others data.
von Willebrand Factor (vWF) is a multimeric glycoprotein that consists of disulfide-bridge linked dimers. It is usually circulating in plasma as a stable complex with the coagulation factor VIII. The human vWF, as a drug product, is a complex that also contains the coagulation factor VIII. This complex was developed by Octapharma Pharmazeutika Produktionsges and FDA approved on August 21, 2015.
The efficacy in clinical trials of vWF in surgical procedures is of 96.7% regardless the type of von Willebrand disease. In a perioperative setting, the hemostatic effects were rated as excellent in a dose of 848.6 IU/kg and no anti-factor VII or inhibitors were detected. These excellent results come from the re-establishment of platelet-adhesion to the subendothelium at sites of vascular damage and the aggregation of platelets. vWF allows the protection of factor VIII from the rapid degradation and thus it restores normal coagulation.
| Trade Name | Von Willebrand Factor (human) |
| Generic | Von Willebrand factor human |
| Von Willebrand factor human Other Names | Factor con Willebrand, Factor von Willebrand, Human von willebrand factor, Von willebrand factor, Von willebrand factor (human), Von willebrand factor complex (human), von Willebrand factor, human, Von willebrand's factor |
| Type | |
| Weight | 20000000.0 Da (All multimers included) |
| Protein binding | Vonicog alfa presents a very high plasma protein binding as its main function is performed in the blood. |
| Groups | Approved, Investigational |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
Von Willebrand Factor (human) is a complex of von Willebrand Factor and Factor VIII used to treat bleeding episodes in von Willebrand disease.
The vWF and Factor VIII complex is indicated for the prevention of excessive bleeding during and after minor and major surgery in adult and pediatric von Willebrand disease patients. It is also indicated for the on-demand treatment and control of bleeding episodes. The von Willebrand disease is an inherited disorder characterized by the deficiency or misfunction of the von Willebrand factor (vWF). Due to this deficiency, the blood cannot clot properly and the patients that present this disease are prone to prolonged or excessive bleeding. There are three types of this disease, and type 3 is an autosomal recessive inherited disorder marked by very low or absent levels of vWF.
Von Willebrand Factor (human) is also used to associated treatment for these conditions: Bleeding, Hemorrhagic episodes
How Von Willebrand Factor (human) works
The vWF is involved in primary and secondary homeostasis and it is also a carrier and stabilizing protein that protects the coagulation factor VIII from proteolysis and clearance. vWF also presents an adhesive function in which it mediates the binding between platelets and subendothelial tissues. The external human vWF acts as the endogenous vWF and thus, it presents all the functions abovementioned.
Toxicity
The administration of human vWF seems to be very well accepted.
Volume of Distribution
The volume of distribution of the human concentrate vWF is 69.7 ml/kg.
Elimination Route
According to clinical trials in patients with von Willebrand disease, the AUC, Cmax and mean residence time was approximately 1119 h.IU/dL, 76 IU/dL and 20.6 hours, respectively.
Half Life
The reported terminal half-life of the concentrate of vWF and factor VIII is of 15.8 hours.
Clearance
The clearance rate of the human concentrate vWF is 3.9 ml.h/kg.
Elimination Route
The majority of vWF is targeted to the liver and spleen which indicates an active regulatory elimination mechanism. It seems to be uptaken mainly by macrophages.
Innovators Monograph
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