OBJECTIVES: A systematic review was conducted to identify monetary costs data associated with adolescents and adults with hemophilia A and B (with or without inhibitors or in mixed population of with and without inhibitors) in the US and EU5 (France, Germany, Italy, Spain, UK), to use in future economic evaluations in these territories.

METHODS: Nine publication databases and five other information sources were searched in June 2022. Two reviewers independently assessed records against the eligibility criteria reported in the review protocol. One reviewer extracted data from eligible studies and a second checked each data point. The review was limited to studies published since 2016 to identify current data.

RESULTS: Forty publications from 31 studies were included. Fourteen studies were primary costing studies; eleven cost-effectiveness analyses, six cost-modelling studies. Thirty studies considered the third- payer’s perspective, however, only five studies reported non-medical costs. Eight studies reported indirect costs. Published costs evidence primarily considered hemophilia A without inhibitors, this was not as widely available for hemophilia B and patients with inhibitors. The annual costs in patients with hemophilia can be higher than $750,000 in the US and €800,000 in the EU5. The majority of costs are related to clotting factors administered either as prophylaxis or on demand, and costs increase with disease severity. Whilst comparative data have not been identified in publications from 2016 to date, published data suggest costs may be higher for patients with inhibitors than without inhibitors. Costs do not appear to vary depending on whether a patient has hemophilia A or B.

CONCLUSIONS: Most studies reporting costs evidence in hemophilia took a third payer perspective only. Costs are mainly related to clotting factor treatments and severity of the disease. Costs data from hemophilia A studies could be considered generalizable to hemophilia B in economic modelling if needed.