Álvarez-Román, MTCarvalho, MBenítez-Hidalgo, OCatarino, CGarcía-Frade, LJRodríguez-López, MAntunes, MFernández-Mosteirín, NKjöllerström, PMorais, SGalmes, BLópez-Fernández, MFRiera-Sans, LRodrigues, JFJiménez-Yuste, VSalvado, R2024-08-132024-08-132023Sangre (Eng). 2023;42(2):47-58http://hdl.handle.net/10400.17/4978Introduction: Treatment options for patients with hemophilia B (PWHB) have improved in the last decade, but epidemiological studies are required to optimize clinical management and define unmet needs. Objective: The objective of the study is to investigate current disease management and quality of life (QoL) in PWHB in Spain and Portugal. Methods: Epidemiological, cross-sectional, multicenter study with moderately severe and severe PWHB. Sociodemographic, epidemiological, clinical, treatment, and QoL data were collected retrospectively over a 24-month period. Results: Of the 75 patients included in the study, 78.7% received prophylactic treatment and 21.3% on demand; 65.3% were treated with SHL-FIX and 10.7% with rIX-FP. Bleeding occurred in 81.3%, mainly in the form of spontaneous bleeding. Prophylaxis resulted in a lower annualized bleeding rate, better joint health, and better QoL, compared to on-demand treatments. Patients treated with rIX-FP throughout the study had better disease control and QoL than those treated with SHL-FIX. Conclusion: With the largest sample to date of PWHB from Spain and Portugal, our understanding of the management and impact of hemophilia B is broadened and, in accordance with previous evidence, it shows the benefits of prophylaxis and the advantages that the FIX extended half life products life FIX can offer.engHemophilia BEpidemiologic studiesTreatment outcomeQuality of lifeSpainPortugalHDE HEM PEDjournal article