OBJECTIVES: Hemophilia B is an inherited bleeding disorder characterized by a deficiency in clotting factor IX (FIX). Its severity varies based on the degree of FIX deficiency, with the most severe cases experiencing frequent spontaneous hemorrhages (FIX levels < 1 IU/dL). Treatment options include on-demand administration following a hemorrhage or prophylaxis to prevent bleeding. A complication is the production of inhibitory antibodies against the coagulation factors in a few patients, leading to more aggressive and expensive treatments. The aim of this study was to estimate the healthcare consumption and related costs of Hemophilia B according to severity in France in 2021.

METHODS: Data relating to all patients diagnosed with Hemophilia B were extracted from the nationwide French claims database (SNDS) for the year 2021.

RESULTS: 1,311 patients with Hemophilia B were identified. Mean age was 36 years and 83.5% were males. The mean annual direct medical costs (drugs, consultations, hospitalization, ...) varied strongly according to treatment modalities and presence of inhibitors:

• €6,506 for 996 patients treated on demand without inhibitors (76.0% of patients)
• €147,677 for 6 patients treated on demand with inhibitors (0.5% of patients)
• €162,635 for 303 patients treated in prophylaxis without inhibitors (23.1% of patients)
• €471,439 for 6 patients treated in prophylaxis with inhibitors (0.5% of patients).

CONCLUSIONS: The cost of Hemophilia B is high, varies greatly with disease severity and presence of inhibitors, and is mostly due to the antihemophilic drugs.