Characterization of Portuguese Haemophilia patients based on the National Registry Data

Abstract

Abstract Haemophilia is a chronic and rare congenital bleeding disorder requiring treatment for life. An updated registry of data on this disease is of paramount importance for documenting prevalence, planning care, and evaluating effectiveness of resources in any country. The study presented in this paper is aimed at characterizing Portuguese patients’ clinical condition and demography, as well as the replacement therapy used in their treatment, based on hemo@record data, a technological solution that supports the National haemophilia registry. The analysis of 110 patients records confirmed the high prevalence of haemophilia A (75.5%) compared with haemophilia B (11.8%) and a large incidence in the severe levels (or the existence of people with mild severity without diagnosis and, consequently, without proper treatment) promoting insight about the portrayal of haemophilia in Portugal. This study should be extended as the registry evolves fostering an ever more efficient management of resources and ultimately a better quality of care for people with haemophilia.