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©The Author(s) 2016.
World J Hematol. Aug 6, 2016; 5(3): 61-74
Published online Aug 6, 2016. doi: 10.5315/wjh.v5.i3.61
Published online Aug 6, 2016. doi: 10.5315/wjh.v5.i3.61
1 Inherited VWD caused by genetic mutations at the VWF locus includes a broad spectrum of recessive and dominant variants of VWD |
2 WD Type 1 is quantitative deficiency of VWF mainly based on a normal VWF:RCo/VWF:Ag ratio. Type 2 VWD is a qualitative deficiency of VWF as documented by a decreased VWF:RCo/VWF:Ag ratio. Type 3 refers to virtually complete deficiency of VWF |
3 VWD Type 2 refers to qualitative variants with absence of high molecular weight VWF multimers and distinguishes 2A (IIA, IIB, IIE, and IID) 2B, 2M and 2 N |
4 VWD Type 2M or 2U is a distinct entity with decreased platelet dependent function (VWF:RCo) and presence of large VWF multimers |
5 VWD Type 2A (IIA, IIC, IIE and IID) refers to qualitative variants with absence of HMW multimers, normal or decreased RIPA and decreased VWF: VWF:RCo/VWF:Ag ratio |
6 VWD Type 2B is a qualitative variant with absence of HMW multimers, decreased VWF:RCo/VWF:Ag ratio and increased RIPA |
7 VWD Type 2N is a mild hemophilia due to FVIII binding defect of VWF, presence of large VWF multimers, normal VWF:RCo/VWF:Ag ratio and decreased FVIII/VWF:Ag ratio |
- Citation: Michiels JJ, Batorova A, Prigancova T, Smejkal P, Penka M, Vangenechten I, Gadisseur A. Changing insights in the diagnosis and classification of autosomal recessive and dominant von Willebrand diseases 1980-2015. World J Hematol 2016; 5(3): 61-74
- URL: https://www.wjgnet.com/2218-6204/full/v5/i3/61.htm
- DOI: https://dx.doi.org/10.5315/wjh.v5.i3.61