Maladie de Willebrand

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by a genetic defect (autosomal transmission, mostly dominant) of the concentration, structure or function of von Willebrand factor (VWF), a multimeric glycoprotein involved in both primary hemostasis (mediator of platelet adhesion and aggregation) and coagulation (carrier protein for factor VIII (FVIII)). VWD is characterized by a great clinical, biological and molecular heterogeneity secondary to the complex structure and functions of VWF. The prevalence of all forms of VWD is about 1% symptomatic forms (ble 0.01%. The diagnosis bleedings±visceral an (panel of miscellaneou 278 Tires a part : A. Veyradier d:10. Pour citer cet article : Veyradier A, Fressinaud E, Goudemand J, Meyer D. La maladie de Wille C op yr ig ht © 2 01 6 Jo hn L ib be y E ur ot ex t. D ow nl oa de d by a r ob ot c om in g fr om 1 57 .5 5. 39 .9 7 on 2 7/ 06 /2 01 6. of the general population while the prevalence of the most eding episodes leading to a hospital management) is about of VWD relies on both clinical symptoms (skin and mucosal d joins bleeding in the most severe form) and biological data s phenotypic and genotypic testing) which allow identifying 164/hm a.21.0629 Hematologie, vol. 17, n o 4, juillet-aout 2011 brand. Hematologie 2011 ; 17 (4) : 278-88 doi:10.1684/hma.2011.0629