Enfermedad de Von Willebrand: tipo III, una revisión de la literatura

Abstract

Von W illebrand's disease is characteristic for its clinical manifestations caused by the alteration of the quantity or structural m orphology of the von Willebrand factor, in type 3 there is an almost total quantitative absence of vWF, the objective of this work is to carry out a bibliographic search about pathophysiology, genetics, clinical picture, diagnosis and therapeutic management in the second level of care; based on scientific information found in articles, updated guidelines, physical and virtual documents that have information on von Willebrand disease using different databases such as: UptoDate, Elsevier, Pubmed, ProQuest, Stat Pearls, National Library of medicine, Scielo, Stat Pearls, Google Scholar, using for search: "Von Willebrand Diseases", "Von Willebrand Diseases/classification", "Von Willebrand-Diseases/complications", "Von Willebrand Diseases/epidemiology" "Von Willebrand disease" published 3-5 years ago in English and Spanish. VWD type 3 is the most severe manifestation that is phenotypically expressed in mucosal, soft tissue, joint, and muscle haemorrhages. For its diagnosis, in addition to the clinic, blood tests are needed where thrombocytopenia, microcytic anemia, prolonged partial thromboplastin time will be evidenced, for treatment, symptomatic management is performed with VWF concentrates and factor VIII.