RESUMEN
Little is known about the challenging treatment of pediatric patients with hemophilia B and inhibitors due to disease rarity. We describe three patients diagnosed in childhood and followed up to 9 years. All three had allergic reactions to Factor IX, but two were later safely treated for bleeding episodes with activated prothrombin complex concentrates (APCC = FEIBA). The third was given only recombinant activated Factor VIIa. Based on ex vivo thrombin generation analysis, a new alternative treatment of combined bypassing agents was administered for bleeding episodes and several minor surgical procedures with no treatment-associated adverse events or thrombosis.
Asunto(s)
Factores de Coagulación Sanguínea/uso terapéutico , Factor VIIa/uso terapéutico , Hemofilia B/tratamiento farmacológico , Trombina/biosíntesis , Adolescente , Pruebas de Coagulación Sanguínea/métodos , Preescolar , Humanos , Masculino , Proteínas Recombinantes/uso terapéutico , Trombina/efectos de los fármacosRESUMEN
A 6 months old infant, diagnosed with a rare mutation causing severe hemophilia A, presented with spinal epidural hematoma. Parents later admitted the infant had glass cupping therapy performed within 2 weeks of the onset of symptoms. The rare mutation, rare bleeding complication, and the eventual course of therapy applied in this case will be discussed in our case report.