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 time consuming and technically challenging method lacking for standardization and available only in few specialized laboratories.
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About
von Willebrand disease (VWD), can be divided into three major types.
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 hemorrhaging after childbirth. The choice of an appropriate therapy depends on VWD subtyping and severity.
About
von Willebrand disease (VWD), can be divided into three major types.
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 hemorrhaging after childbirth. The choice of an appropriate therapy depends on VWD subtyping and severity.
Technical characteristics
- Tube capacity: from 120 samples with the onboard loading
- 24/7 accessibility
- Automatic start-up / shut down
- Automatic maintenance
- Main reagents compartment with 4 flexible positions for multiple buffers
- Secondary reagent deck with 13 flexible positions for additional reagents
- Cooled section in the secondary reagent deck
- Smart LCD colored touch screen with user friendly interface
- Color-coded back lighted reagent containers
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“1 Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost. 2006;4:2103–14.
2. Budde U, Schneppenheim R, Plendl H, Dent J, Ruggeri ZM, Zimmerman TS. Luminographic detection of von Willebrand factor
multimers in agarose gels and on nitrocellulose membranes. Thromb Haemost. 1990;63:312–5.
3. Schneppenheim R, Plendl H, Budde U. Luminography–an alternative assay for detection of von Willebrand factor multimers.
Thromb Haemost. 1988;60:133–6.
4. Vangenechten I, Gadisseur, Improving diagnosis of von Willebrand disease: Reference
ranges for von Willebrand factor multimer distribution, Res Pract Thromb Haemost, 2020 Jul 16;4(6):1024-1034.”
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