Emicizumab
Biochemistry
| Classification | Humanized bispecific antibody of the IgG type |
|---|---|
| Molecular weight | App. 145.000 Da |
| Production | Genetic production |
| Function | Emicizumab binds to both Factor IXa and Factor X and mediates the activation of Factor X to Factor Xa via Factor IXa. It bridges the activated Factor IXa with the Factor Xa and thus mimicks the cofactor function of FVIII. |
Clinical Significance
Emicizumab is used as a routine prophylaxis to reduce or prevent bleeding episodes in patients with hemophilia A and factor VIII inhibitors and in patients with severe haemophilia without factor VIII inhibitors. It can be applied to all age groups. (as of December 2020)
The bispecific antibody restores the function on factor VIII, whose activity is reduced or completely absent in hemophilia-A-patients. Since emicizumab imitates the cofactor activity of factor VIII, it thus acts as a factor VIII mimetic.
Indication
- Bleeding complications despite calculated adequate substitution
- Preoperative level determination
Literature
- Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, Santagostino E, Kruse-Jarres R, Negrier C, Kessler C, Valente N, Asikanius E, Levy GG, Windyga J, Shima M. Emicizumab Prophylaxis in Hemophilia A with Inhibitors N; Engl J Med. 377(9): 809-818, 2017.
- Barnett B, Kruse-Jarres R, Leissinger CA. Current management of acqured factor VIII inhibitors. 15(5): 451-5, 2018.
- Jan Astermark. FVIII inhibitors: pathogenesis and avoidance; Blood 125:2045-205, 2015.
- Wittmer C, Young G. FVIII Inbibitors in Hemophilia A: rationale and latest evidence; Ther. Adv. Hematol. 4: 59-72, 2013.
- Hemlibra Summary of product characteristics (retrieved 22.02.2021)