Hemofilia a y b: manejo del paciente con inhibidores

Abstract

Hemophilia is an affection defined by a hereditary and congenital hemorrhagic alteration in which the blood does not coagulate properly, causing hemorrhages spontaneously or after witnessing an injury or surgical procedure, mainly manifested by mutations in the chromosome X, in which there is a decrease or absence of the activity of factors VIII or IX, within this pathology inhibitors can be produced, these being alloantibodies that act against coagulation factor VIII and IX, which are responsible for the suspension of bleeding, the presence of inhibitors can be suspected when the bleeding does not stop with the usual dose of treatment, which are the concentrates of said factors. In this review, sources that contain verified and real information were investigated in publications of scientific journals such as The New England Journal of Medicine, The official Journal of the World Federation of Hemophilia, Haematologica, WHO and databases such as Scopus, PubMed, The Cochrane, Google Scholar, Uptodate, BMC Medicine with a period of time between 2016 and 2021 in the Spanish and English languages. This pathology requires an interdisciplinary, complex and often costly management. Treatment depends on the location and severity of the bleeding, the titer and the characteristics of the inhibitor. Among the drugs used to manage this pathology are recombinant activated FVII rFVIIa, activated prothrombin complex concentrates (APCC) and the use of Rituximab and Emicizumab