von Willebrand Disease Reflexive Profile
Use
vWF is produced by megakaryocytes and endothelial cells.2 The vWF protein polymerizes in plasma into multimers of up to 100 subunits that range in size from 0.5 to 20 million daltons.1,2 vWF's plasma half-life is about 24 hours. vWF performs two major functions in hemostasis.1,2 vWF acts a cross-linking protein that connects platelets to each other and to subendothelial cells at the site of vascular damage. This functionality is dependent on the presence of high molecular weight multimers of vWF. vWF also serves as an obligate carrier of factor VIII in plasma. Functional vWF serves to protect factor VIII from proteolytic degradation and effectively increases its half-life fivefold.4 Effective factor VIII binding is not dependent on the presence of large multimeric vWF complexes.3 The assessment of vWF activity requires two tests to evaluate these two functions. The vWF activity (ristocetin cofactor) assay reflects the effectiveness of the patient vWF in supporting platelet adhesion. Factor VIII levels can be diminished due to low vWF levels or due to ineffective factor VIII binding by a defective vWF.
Special Instructions
If a reflex test is performed, additional charges/CPT code(s) may apply. Plasma must be separated from cells within 45 minutes of venipuncture and centrifuged a second time before being placed in plastic transport tubes.
Limitations
The tests include von Willebrand Factor Activity and Antigen, which are FDA approved. However, the CBA (Collagen Binding Activity) is for research use only (RUO), and the von Willebrand Multimeric Analysis is a laboratory developed test (LDT). The performance characteristics of the vWF Collagen Binding Activity test are not established for clinical diagnosis without confirmation from another diagnostic method.
Methodology
Other
Biomarkers
LOINC Codes
- 48593-8
- 6014-5
- 27816-8
- 3209-4
- 21026-0
Result Turnaround Time
4-5 days
Related Documents
For more information, please review the documents below
Specimen
Plasma
Volume
2 x 2 mL
Minimum Volume
2 mL
Container
Blue-top (sodium citrate) tube
Collection Instructions
Plasma must be separated from cells within 45 minutes of venipuncture and centrifuged a second time before being placed in plastic transport tubes. Freeze within two hours and keep frozen until testing is performed.
Storage Instructions
Freeze.
Stability Requirements
| Temperature | Period |
|---|---|
| Frozen | 2 months |
