von Willebrand Factor (VWF) Multimers

Coagulation

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Description

von Willebrand disease (VWD) is a bleeding disorder caused by a reduction or dysfunction of Von Willebrand factor (VWF). VWF is a large multimeric glycoprotein circulates in the plasma as a series of polymers called multimers. Multimers are a variable number of subunits (500-more than 10,000 kDa) linked by disulphide bonds. The multimers may be steadily released from endothelial cells or stored in Weibel-Palade bodies in platelets. High molecular weight multimers are important for binding to collagen and platelet receptors during primary haemostasis to facilitate wound healing. Multimer analysis is Sheffield is initially by a semi-automated multimer assay, the Hydragel 5 von Willebrand multimer method, which utilises Hydrasys 2 Scan instrumentation to perform agarose gel electrophoresis using preformed 2% agarose gels, direct immunofixation, visualization with peroxidase‐labelled antibody, and densitometry. Further investigation may be performed by our in-house agarose gel electrophoresis method if required.

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Indications

VWD is classified into the quantitative type 1 disorder, the qualitative type 2 disorders (A, B, M, N) and type 3.which is an absence of VWF. Multimer analysis will enable confirmation and subtyping of VWD in patients suspected of the disorder. An overall loss of multimers is observed in type 1 VWD, a loss of high (and in some cases, intermediate) molecular weight multimers is observed with type 2A and type 2B VWD, a normal multimer pattern is observed in types 2M and 2N VWD and an absence of multimers is observed in type 3 VWD.

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Sample Type

Plasma (Citrate Blue) x 2.

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Reference Range

Interpretation on report.

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Turnaround Time

Within 9 weeks

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Testing Frequency

As required (usually batched once a month).

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External Notes

Contact the laboratory or Haematology registrar to discuss appropriate tests.

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References

Laffan M et al (2004). The diagnosis of VWD. A guideline from the UK HDCO. Haemophilia 10, 199-217
Ruggeri ZM et al (1981). The complex multimeric composition of VWF. Blood, 57, 1140-43
Bowyer, AE, Goodfellow KJ et al (2018). Evaluation of a semi‐automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers. Res Pract Thromb Haemost, 2 (4), 790-799

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See Also

VWF Antigen (VWF:Ag)
VWF:FVIII Binding Assay

Please note: the above information is subject to change and we endeavour to keep this website up to date wherever necessary.