Objectives. Haemophilia A is a congenital disorder of coagulation that mainly affects males and causes a considerable use of
resources, especially when hemophilic patients are treated with prophylaxis. The aim of the present review was to discuss and
appraise the methodological aspects and results of published economic evaluations of haemophilia A treatments in the last
decade. Methods.The literature search, performed by consulting four engines, covered studies published between 2002 and 2014.
Full economic evaluations published in English language were identified and included in the review. A quality assessment of
the studies was also carried out based on Drummond’s checklist. Results. After careful evaluations of the identified records, 5
studies were reviewed. Primary and secondary prophylaxis resulted cost-effective compared to on-demand therapy: the ICER of
primary prophylaxis ranged from C40.236 to C59.315/QALY gained, while the ICER of secondary prophylaxis was C40.229/QALY
gained. Furthermore, 60% were high quality and 40% were medium quality studies. Conclusions.The review underlines the cost-effectiveness
of prophylaxis versus on-demand treatment and the different methodological approaches applied. Further economic
evaluations are required with models that reflect the clinical reality and consumption of resources in each countr
