Test ID: VWFM2
von Willebrand Factor Multimer Analysis, Plasma
NY State Approved 
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Useful For Suggests clinical disorders or settings where the test may be helpful
Subtyping of von Willebrand factor:
-When results of complementary laboratory tests (eg, F8A/9070 Coagulation Factor VIII Activity Assay, Plasma; VWFX/89792 von Willebrand Activity, Plasma; and VWAG/9051 von Willebrand Factor Antigen, Plasma) are abnormally low or discordant.
-This test is primarily used to identify variants of type 2 von Willebrand factor.
-As an aid determining appropriate treatment
Special Instructions and Forms Describes specimen collection and preparation information, test algorithms, and other information pertinent to test. Also includes pertinent information and consent forms to be used when requesting a particular test
Method Name A short description of the method used to perform the test
Agarose Gel Electrophoresis/Infrared Dye-Labeled Antibody Detection
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Aliases Lists additional common names for a test, as an aid in searching
Von Willebrand Protein Polymers sDS Gel, Plasma
VWF Mult Anal - Off hours order 399
Specimen Type Describes the specimen type needed for testing
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
VWFX/89792 von Willebrand Factor Activity, Plasma and VWAG/9051 von Willebrand Factor Antigen, Plasma are required before performing this test. If already assayed, submit results. If no results are included, submit separate specimens for the above assays following specimen requirements for each test.
See Coagulation Studies in Special Instructions.
Specimen Type: Platelet-poor plasma
Collection Container/Tube: Light-blue top (citrate)
Submission Container/Tube: Plastic vials
Specimen Volume: 1 mL in 2 plastic vials each containing 0.5 mL
Collection Instructions:
1. Specimen should be drawn prior to coagulation factor replacement therapy.
2. Spin down, remove plasma, and spin plasma again.
3. Freeze specimens immediately at < or =-40 degrees C, if possible.
4. Send specimens in the same shipping container.
Additional Information:
1. Double-centrifuged specimen is critical for accurate results as platelet contamination may cause spurious results.
2. Each coagulation assay requested should have its own vial.
3. Coagulation testing is highly complex, often requiring the performance of multiple assays and correlation with clinical information. For that reason, we suggest ordering VWPR/83099 von Willebrand Profile.
Forms: Coagulation Patient Information Sheet (Supply T675) in Special Instructions
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Reject Due To Identifies specimen types and conditions that may cause the specimen to be rejected
| Hemolysis | Mild OK; Gross reject |
| Lipemia | Mild OK; Gross reject |
| Icterus | Mild OK; Gross reject |
| Other | NA |
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
| Specimen Type | Temperature | Time |
|---|---|---|
| Plasma Na Cit | Frozen | 42 days |
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
von Willebrand factor (VWF) is a large multimeric plasma glycoprotein that performs 2 critical functions in hemostasis:
-VWF is a ligand and mediates platelet adhesion to the subendothelial matrix at the site of vessel wall injury by binding to the constitutively active platelet receptor glycoprotein (GP)-Ib, V, IX complex, and to subendothelial matrix collagen
-VWF is a carrier molecule for procoagulant factor VIII in the circulation, increasing the factor VIII half-life 5-fold. Under conditions of high shear, VWF also mediates platelet-platelet cohesion by binding to the platelet receptor GP-IIb/IIIa (integrin alpha IIb beta3)
A bleeding disorder, von Willebrand disease (VWD), occurs when VWF is quantitatively deficient or qualitatively abnormal. VWD manifests clinically as easy bruising, mucocutaneous bleeding (eg, epistaxis, menorrhagia), and bleeding after trauma or surgery. VWD is the most common of the inherited bleeding disorders, and can also occur on an acquired basis.
Plasma VWF consists of a series of multimers varying in size from dimers to multimers over 40 subunits (>10 million Daltons). The largest multimers provide multiple binding sites that can interact with both platelet receptors and subendothelial matrix sites of injury, and are the most hemostatically active form of VWF.
Inherited VvWD has been classified into 3 types:
-Type 1, typically an autosomal dominant disease, is the most common, accounting for approximately 70% of VvWD patients. It represents a quantitative deficiency of VWF of variable severity.
-Type 2, which is usually an autosomal dominant disease, is characterized by several qualitative abnormalities of vFWVFW . Four subtypes have been identified: 2A, 2B, 2M, and 2N.
-Type 3, an autosomal recessive disorder, leads to severe disease with extremely reduced or undetectable levels of VWF, as well as very low levels of factor VIII.
Acquired von Willebrand syndrome (AVWS) is associated with a number of different disease states and is caused by several different pathophysiological mechanisms, including antibody formation, proteolysis, binding to tumor cells with increased clearance, and decreased synthesis. AVWS is most frequently described in patients with dysproteinemias (including monoclonal gammopathy of undetermined significance [MGUS], multiple myeloma, and macroglobulinemia), lymphoproliferative disorders, myeloproliferative disorders (eg, essential thrombocythemia), autoimmune diseases (eg, systemic lupus erythematosus), severe aortic stenosis, gastrointestinal angiodysplasia, and hypothyroidism.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
An interpretive report will be provided.
Interpretation Provides information to assist in interpretation of the test results
The plasma von Willebrand factor (VWF) multimer analysis is a qualitative visual assessment of the size spectrum and the banding pattern of VWF multimers.
This test is used to identify variants of type 2 von Willebrand disease that have fewer of the largest multimers, have unusually large multimers, or have qualitatively abnormal "bands" that indicate an abnormal VWF structure.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
von Willebrand factor (vWF) multimer analysis is not useful if:
-The following tests are normal
- F8A/9070 Coagulation Factor VIII Activity Assay, Plasma
- VWFX/89792 von Willebrand Factor Activity, Plasma
- VWAG/9051 von Willebrand Factor Antigen, Plasma
-The vWF activity:vWF antigen ratio is > or =0.8
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
1. Budde U, Schneppenheim R: von Willebrand Factor and von Willebrand Disease. Rev Clin Exp Hematol 2001;5.4:335-368
2. Ruggeri ZM: Structure and function of von Willebrand factor: Relationship to von Willebrand’s disease. Mayo Clinic Proc 1991;66:847-861
3. Sadler JE: A revised classification of von Willebrand disease. Thromb Haemost 1994;71:520-525
4. Laffan M, Brown SA, Collins PW, et al: The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors Organization. Haemophilia 2004;10:199-217
5. Mannucci PM: Treatment of von Willebrand’s disease. N Engl J Med 2004;351:683-694
6. Pruthi RKl: Plasma von Willebrand factor multimer quantitative analysis by in-gel immunostaining and infrared fluorescent imaging. Thromb Res 2010;126:543-549
Method Description Describes how the test is performed and provides a method-specific reference
Platelet-poor plasma proteins are denatured using heat and an anionic detergent, sodium dodecyl sulfate. The sample is then electrophoresed through a discontinuous agarose gel on a cooled horizontal electrophoresis unit overnight to separate the von Willebrand factor (VvWF) multimers by size. The gel is fixed in acid and isopropanol, washed in water, and incubated with dilute rabbit antihuman VvWF. After washing away unbound antibody, the gel is incubated with dilute goat antirabbit IgG antibody tagged with an infrared dye. Excess secondary antibody is washed away, and the gel is scanned using an infrared imaging system. The digitized image of the electrophoretic distribution of the vWF multimers is interpreted by a coagulation consultant and a written report is provided. (Ruggeri ZM, Zimmerman TS:, Variant von Willebrand's disease. Characterization of two subtypes by analysis of multimeric composition of FVIII/vWF in plasma and platelets. J Clin Invest 1980;65:1318-1325)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday, Tuesday; 9 a.m.-4 p.m.
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
Test Classification Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
CPT Code Information Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
85247
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
| Result ID | Reporting Name | LOINC Code |
|---|---|---|
| 8844 | von Willebrand Factor Multimer, P | 32217-2 |
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