von Willebrand Disease Panel with Collagen Binding Assay (CBA)

Test Code

VWDP2

Quest Code

15540

CPT Codes
83520, 85240, 85245, 85246, 85247, 85730

Includes

Partial Thromboplastin Time, Activated
Factor VIII Activity, Clotting
von Willebrand Factor Antigen
Ristocetin Cofactor
von Willebrand Factor Collagen Binding Assay
von Willebrand Antigen, Multimeric Analysis

Preferred Specimen

1 mL plasma collected in each of four separate 3.2% sodium citrate (light blue-top) tubes

Minimum Volume

0.75 mL (x4)

Instructions

Please submit a separate, frozen vial for each special coagulation assay ordered. Draw blood in a light blue-top tube containing 3.2% sodium citrate, mix gently by inverting 3-4 times. Centrifuge 15 minutes at 1500 g within one hour of collection. Using a plastic pipette, remove plasma, taking care to avoid the WBC/platelet buffy layer and place into a plastic vial. Centrifuge a second time and transfer platelet-poor plasma into a new plastic vial(s). Freeze immediately and transport on dry ice.

Note: Storage of whole blood at refrigerated temperatures prior to processing may lead to cryoprecipitate formation and falsely low Factor VIII and von Willebrand Factor studies.

Transport Temperature

Frozen

Specimen Stability

Room temperature: Unacceptable
Refrigerated: Unacceptable
Frozen: 14 days

Reject Criteria (Eg, hemolysis? Lipemia? Thaw/Other?)

See individual assays

Methodology
See individual tests

FDA Status
von Willebrand Factor Collagen Binding Assay: This test was performed using a kit that has not been cleared or approved by the FDA. The analytical performance characteristics of this test have been determined by Quest Diagnostics. This test should not be used for diagnosis without confirmation by other medically established means.

von Willebrand Antigen, Multimeric Analysis: This test was developed and its analytical performance characteristics have been determined by Quest Diagnostics. It has not been cleared or approved by FDA. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.

Setup Schedule

Set up and Report available: See individual assays

Reference Range

See Laboratory Report

Clinical Significance

This test may be used to help diagnose von Willebrand disease (VWD) and categorize disease subtype.

von Willebrand factor (VWF) is a blood coagulation protein that has 2 functions: (1) mediating platelet adhesion to the injured endothelium and (2) acting as a protective carrier protein for coagulation factor VIII. Deficiency of VWF causes VWD, the most common inherited bleeding disorder that affects approximately 1 in 1,000 individuals at the primary care level [1]. VWD is classified into type 1 (quantitative deficiency of VWF), type 2 (qualitative deficiency of VWF), and type 3 (total absence of VWF). Different types of VWD are further categorized into subtypes, identification of which is important to inform prognosis and treatment decisions.

Three tests are recommended for initial diagnosis of VWD [2]: VWF antigen, platelet-dependent VWF activity (eg, ristocetin cofactor), and factor VIII activity. Additional tests may be needed to further identify the subtype of VWD. VWF multimer analysis and collagen binding activity are included in this panel and are useful to discriminate between VWD subtype 2A/2B from 2M and rare forms of 2M [1].

Test results of acquired von Willebrand syndrome (AVWS) may be similar to those of congenital VWD [1, 2]. However, patients with AVWS do not have a family history of bleeding or a personal life-long history of bleeding. Conditions associated with AVWS include lymphoproliferative disorders, monoclonal gammopathy of undetermined significance, Wilms tumor expressing GPIb, myeloproliferative disorders, aortic stenosis, ventricular assist devices, and hypothyroidism.

The results of this test should be interpreted in the context of pertinent clinical and family history and physical examination findings.

References
1. James PD, et al. Blood Adv. 2021;5(1):280-300.
2. Higgins RA, et al. Platelets and van Willebrand factor. In: Rifai R, et al, eds. Tietz Textbook of Laboratory Medicine. 7th ed. Elsevier Inc; 2022.

Performing Laboratory
Quest Diagnostics Nichols Institute
14225 Newbrook Drive
Chantilly, VA 20153

Last Updated: January 28, 2020

The CPT Codes provided in this document are based on AMA guidelines and are for informational purposes only. CPT coding is the sole responsibility of the billing party. Please direct any questions regarding coding to the payor being billed. Any Profile/panel component may be ordered separately. Reflex tests are performed at an additional charge.