Abstract
Von Willebrand factor (vWf) is a fundamental multimeric plasma glycoprotein in the coagulation process. Its function is to mediate platelet adhesion and to stabilize circulating factor VIII. A functional or quantitative alteration of vWf gives rise to von Willebrand disease (vWD). The association between vWD and angiodysplasia was described in 1967, but it was only until 2011 that Starke et al demonstrated the in vitro and in vivo role of vWf in angiogenesis. Congenital or acquired vWf deficiency, especially of high molecular weight multimeters, not only favors bleeding, but also contributes to increased angiogenesis in these patients. The treatment should be focused both on the control of the acute episode of gastrointestinal bleeding, with vWf replacement therapy and local endoscopic treatment, as well as on the prevention of the progression of angiodysplasia and future bleeding. There are different published therapeutic approaches using vWf replacement that are not effective in all patients. Recently, angiogenesis inhibitor medications have been used.
Highlights
El factor de von Willebrand es una glicoproteína multimérica plasmática
Ausente incluso < 0,6 mg/dl vWF:Ag: Factor von Willebrand antigénico; FVIII:C: Factor VIII coagulante; vWF:FVIIIB: Prueba de unión factor von Willebrand-factor VIII; RIPA: agregrometría con dosis crecientes de ristocetina
Persistent gastrointestinal bleeding due to angiodysplasia of the gut in von Willebrand’s disease
Summary
Von Willebrand factor (vWf) is a fundamental multimeric plasma glycoprotein in the coagulation process. A functional or quantitative alteration of vWf gives rise to von Willebrand disease (vWD). El factor de von Willebrand (vWf) es una glicoproteína multimérica plasmática. Su disfunción cuantitativa o cualitativa genera el trastorno hereditario de la coagulación más común, la enfermedad de von Willebrand (vWD). En el aparato de Golgi se forman multímeros y adquieren una conformación tubular en los WPBs, luego, al fusionarse con la membrana plasmática de las células endoteliales, estos multímetros ultralargos son clivados en su dominio A2 por la metaloproteasa ADAMTS13 a hebras más pequeñas que circulan en el plasma[2]. La enfermedad de von Willebrand refleja la disminución de la síntesis del factor, vWD tipo 1 y 3, o su disfunción vWD tipo 2.
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