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Poster Networking

Inherited Bleeding Disorders and Inhibitors

PB0543 - "Experience of switching to EHL-CFVIII or non-replacement therapies in patients with severe or moderate hemophilia a without inhibitor from the galician community: retrospective analysis"

Monday, June 24, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall
Background: Hemophilia treatment has changed in recent years.

Aims: We conducted a retrospective study on patients with severe or moderate Hemophilia A (HA) without inhibitor, who changed from standard half-life factor (SHL) to extended half-life factor (EHL) or emicizumab in Galicia until December 2023.The analysis will cover the 12 months prior to the change and the 12 months following, as well as the last 12 months.

Methods: 27 males were included, with a median age of 29.5 years. Baseline characteristics are shown under Table 1 and Figure 1.

Results: Regarding consumption, the median was 312,000 IU/year (IQR 260,000-410,625) for SHL vs. 266,875 IU/year (IQR 220,000-312,000) for EHL, with a median of 3 infusions/week (IQR 3-3.5) vs. 1.875 infusions/week (IQR 1.4-2.33) respectively. The trough level with SHL was an average of 1.67% (SD 1.14) vs. 3.8% (SD 2.81) for EHL (p=0.034). SHL had an average of 2.33 (SD 2.99) bleeds vs. 0.83 (SD 0.76) for EHL and 0.3 (SD 0.47) in the last 12 months (p < 0.0028 and 0.0002 respectively) with a bleeding reduction rate of 64.28% in the 12 months of the change and 87.5% in the last 12 months. Similarly, an average spontaneous annual bleeding rate of 0.45 (SD 1.02) vs. 0.33 (SD 0.7) vs. 0.1 (SD 0.28) respectively was observed, with a decrease of 27.27% and 81.81% in the last 12 months. The average joint bleed was 1.66 (SD 2.39) with EHL, vs. 0.66 (SD 0.76) vs. 0.13 (SD 0.34) with a reduction rate of 60% and 92.5%. Arthropathy was present in 42.31% and 36.36% after the switch to EHL.

Conclusion(s): An improvement in the clinical profile of the hemophiliac patient can be observed, not only due to the EHL product but also due to the increase in the trough level of the factor, with a lower consumption.