Table 1.
EHL product | Generic name | Mechanism of action | Half-life of EHL product (hours) | Half-life of standard factor (hours) |
Ratio (EHL to standard factor half-life) | Licensing status | Frequency of administration | Comments and notable points |
---|---|---|---|---|---|---|---|---|
FVIII extended half-life products for use in Haemophilia A | ||||||||
Lonoctocog alfa pegol | Afstyla | B-domain truncated single chain | 14.5 | 13.3 | 1.1 | Licensed | Every second day | Only modest improvement in half-life thus requiring more frequent administration than other EHLs |
Efmoroctocog alfa | Elocta | FVIII Fc fusion protein | 19.0 | 12.4 | 1.5 | Licensed | Effective with once-weekly administration | |
Damoctocog alfa pegol | Jivi | PEGylated B-domain deleted FVIII | 18.5 | 13.0 | 1.4 | Licensed | Once weekly administration possible | Not licensed for use in children under 12 |
Rurioctocog alfa pegol | Adynovi | PEGylated FVIII | 14.3 | 10.4 | 1.4 | Licensed | Twice weekly or less | |
Turoctocog alfa pegol | NovoEight | PEGylated B-domain truncated FVIII | 19.5 | 13.0 | 1.5 | Licensed | Every 4–7 days | Can be used in all age groups, including under 12 years |
FIX extended half-life products for use in Haemophilia B | ||||||||
Nonacog beta pegol | Refixia | PEGylated B-domain truncated FIX | 92.7 | 17.8 | 5.2 | Licensed | Currently licenced for administration every 3–4 days | Not licensed for use in children under 12 |
Albutrepenonacog alfa | Idelvion | FIX albumin fusion protein | 92.0 | 17.0 | 5.4 | Licensed | Administration up to once every 14 days (up to 7 days in children) | |
Eftrenonacog alfa | Alprolix | FIX Fc fusion protein | 82.1 | 33.8 | 2.4 | Licensed | Once every 7–10 days in all age groups | |
Dalcinonacog alfa | N/A | rFIX with 3 amino acid mutations | 106.9 | 21.0 | 5.1 | Phase IIb | Currently once daily | The only EHL currently to offer subcutaneous administration |
Information regarding their administration frequencies along with any notable points regarding individual EHLs are also provided
EHL extended half-life; FVIII factor VIII; FIX factor IX