Changing insights in the diagnosis and classification of autosomal recessive and dominant von Willebrand diseases 1980-2015

Figure 7 Translation and integration of the 2006 International Society on Thrombosis and Haemostasis and the 2009 Hamburg classification. VWD type 2 variants[20] (recessive IIC, recessive 2N, dominant IIE, 2B, 2M, IIA, 2CB 1 (sm) and IID related to clustered distribution of VWF gene mutations in the D1-D2 propeptide, D’, D3, A1, A1, A2, A3, D4 and CK domains respectively. For explanation see Figure 8: from left to right recessive 2N, recessive IIC → 2C, and dominant → IIE → 2E, 2B, 2M, IIA → 2A, 2 CB (collagen binding defect), 1 smeary pattern (sm) and IID → 2D (from ref.[20]). VWD: Von Willebrand disease; VWF: Von Willebrand factor.