Palmero‑Picazo J, Rodríguez‑Gallegos MF

Idioma: Español
Referencias bibliográficas: 40
Paginas: 282-287
Archivo PDF: 227.80 Kb.

RESUMEN

La enfermedad de Von Willebrand es el desorden hemorrágico hereditario más común. Se caracteriza por una tendencia a la hemorragia debido a una alteración cuantitativa o cualitativa del factor Von Willebrand. Objetivos: Presentar el cuadro clínico de la enfermedad de Von Willebrand, su etiología, fisiopatología, así como la integración diagnóstica, y tratamiento actualizado. Material y métodos: Estudio observacional descriptivo realizado mediante la búsqueda sistemática de EBSCO y PUBMED, se utilizaron palabras clave como: enfermedad de Von Willebrand, factor Von Willebrand, coagulopatía, fenotipo, genotipo; seleccionando de manera arbitraria los artículos más relevantes en inglés, posteriores al año 2015. Se obtuvieron 40 artículos. Resultados: Existen tres tipos: 1, 2A, 2B, 2N, 2M, y 3. Dentro de su etiopatogenia se muestra una asociación con mutaciones y alteraciones genéticas, principalmente en el cromosoma 12, en la región p13.2, cuya función es poseer el código que servirá para la transcripción y traducción del factor de Von Willebrand. Su diagnóstico es mediante interrogatorio dirigido, estudios de coagulación y la determinación de los multímeros de Von Willebrand. El tratamiento está basado en desmopresina, concentrados de FvW, antifibrinolíticos, estrógenos y transfusiones plaquetarias. Conclusiones: El diagnóstico y tratamiento adecuado es imprescindible para evitar complicaciones y hemorragias mortales, principalmente durante cirugía o procedimientos odontológicos.

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