About

von Willebrand disease (VWD), can be divided into three major types(1):

  • Type 1 is characterized by partial quantitative deficiency of functionally normal VWF and type 3 with a complete absence.

 

  • Type 2 VWD is characterized by a qualitatively defective VWF, which results into an asymmetric decrease in VWF activity and VWF antigen (VWF:Ag).

 

  • Type 2 VWD is subdivided into 2A, 2B, 2M, and 2N based on the specific functional defect(s). A fourth type, acquired VWD, is not hereditary. This type of VWD in adults results after a diagnosis of an autoimmune disease, or from heart disease or some types of cancer. It can also be due to underlying medical disorder.

 

People with VWD experience frequent nosebleeds, easy bruising, and excessive bleeding during and after invasive procedures, such as tooth extractions and surgery. Women often experience heavy menstrual bleeding (heavy menstrual periods that last longer than average) and haemorrhaging after childbirth.

The choice of an appropriate therapy depends on VWD subtyping and severity.

1%
of the population is affected by the VWD. It occurs equally in men and women.
1/10 000
individuals with VWD are symptomatic.

Diagnosis

The diagnosis and typing of VWD requires the combination of several specialized tests. VWF multimers analysis is the only test allowing to detect abnormalities in multimers distribution, important for VWD subtyping and for finding the etiology of acquired VWD. Using electrophoretic protein separation, VWF multimers can be separated into following classes based on their molecular weight (MW): low (LMWM), intermediate (IMWM) and high MW (HMWM).

The VWF multimeric method based on luminographic detection,(2,3) is considered the “gold standard” for diagnosis VWD but the VWF multimers analysis on-homemade gels is a time consuming and technically challenging method, without standardization and available only in few specialized laboratories.

Sebia's expertise

Thanks to our strong know-how in gel electrophoresis techniques, Sebia commercialized the VWF multimers analysis to significantly improve the efficiency of laboratories performing VWF analysis.

HYDRAGEL von WILLEBRAND MULTIMERS assay allows to assess the overall size distribution of von Willebrand factor multimers in human plasma using agarose gel electrophoresis and immunofixation, with the semi-automated the HYDRASYS 2 instrument.