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Haemophilia A is a genetic disorder that causes the absence of FVIII due to mutations in the genes that encode FVIII.
current common treatment for haemophilia A is regular infusion of FVIII, however, patients usually need to receive 3-4 FVIII infusions per week, which bring great inconvenience to their lives.
natural FVIII has a half-life of only about 12 hours in the blood, where it binds to a complex called von Willebrand factor (VWF).
The combination of
VWF and FVIII improves the stability of FVIII in the blood, but the speed at which VWF itself degrades also sets an upper limit on the time FVIII can stay in the blood, because when VWF is degraded, the FVIII combined with it is also degraded.
the design of the BIVV001 connects FVIII to a VWF fragment, the resulting complex does not combine with the natural VWF in the blood, thus breaking the half-life limit set by VWF.
aims to allow haemophilia A patients to achieve near-normal levels of FVIII factor activity for most of the week after receiving an injection.
open label, multi-center study called EXTEN-A I assessed the safety, resistance, and pharmacological dynamics of BIVV001 treatments at doses of 25 IU/kg (n-6) and 65 IU/kg (n-8) in subjects aged 19-63.
test results showed that BIVV001 showed good tolerance and the production of FVIII inhibitors was not detected within 28 days of being given.
no allergic reactions or clinically significant therapeutic adverse events were detected during the study.
in the 65 IU/kg dose queue, BIVV001 single administration reached a 43-hour FVIII half-life, more than three times longer than the 13-hour half-life of traditional recombinant FVIII.
the average coagulation factor VIII activity level was 51% in the 4 days after the first infusion of BIVV001, which was in the normal range, and the activity level of the coagulation factor VIII was 17% after 7 days of infusion.
the average plasma FVIII activity data of patients with haemophilia after receiving a single BIVV001 infusion (Photo source: Reference: Reference 2)) In the 25 IU/kg dose queue, the FVIII half-life of BIVV001 was 38 hours, which was 4 times the 9-hour half-life observed by rFVIII, and the average factor activity level was 5 days after infusion of BIVV001.
references: s1? New England Journal of Medicine publishes positive final results from Phase 1/2a study of BIVV001 in people with severe hemophilia A. Retrieved September 10, 2020, from .2) Konkle et al., (2020). BIVV001 Fusion Protein as Factor VIII Therapy for Hemophilia A. NEJM, DOI: 10.1056/NEJMoa2002699.