Pathophysiology Von Willebrand disease



von willebrand factor active in conditions of high blood flow , shear stress. deficiency of vwf, therefore, shows in organs extensive small vessels, such skin, gastrointestinal tract, , uterus. in angiodysplasia, form of telangiectasia of colon, shear stress higher in average capillaries, , risk of bleeding increased concomitantly.


vwf carries factor vii (fviii), stimulates start of clotting process. typically, individual experience decreased levels of both vwf , fviii.


in more severe cases of type 1 vwd, genetic changes common within vwf gene , highly penetrant. in milder cases of type 1 vwd, complex spectrum of molecular pathology may exist in addition polymorphisms of vwf gene alone. individual s abo blood group can influence presentation , pathology of vwd. individuals blood group o have lower mean level individuals other blood groups. unless abo group–specific vwf:antigen reference ranges used, normal group o individuals can diagnosed type vwd, , individuals of blood group ab genetic defect of vwf may have diagnosis overlooked because vwf levels elevated due blood group.








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