A Phase 3 open-label, multicenter study of the long-term safety and efficacy of intravenous recombinant coagulation factor VIII Fc-von Willebrand factor-XTEN fusion protein (rFVIIIFc-VWF-XTEN; BIVV001) in Previously Treated Patients with severe hemophilia A

For Arm A
- Ability to understand the purpose and risks of the study and provide signed
and dated
informed consent and authorization to use protected health information (PHI) in
accordance with national and local participant privacy regulations. Parents or
legal
guardians* consent is required for participants who are <18 years of age or
unable to give
consent, or as applicable per local laws. Participants who are <18 years of age
may provide
assent in addition to the parents/legal guardians consent, if appropriate.
- Participants who have completed the studies EFC16923, EFC16925, Arm B or Arm
C of
the current study, or any other potential BIVV001 study.
- Male or female: Contraceptive use by men or women should be consistent with
local regulations regarding the methods of contraception for those
participating in clinical studies.
- Willingness and ability of the participant or surrogate (a caregiver or a
family member
>=18 years of age) to continue use of the study ePD throughout the study.

For Arm C
- Participants who have severe hemophilia A, defined as <1 IU/dL (<1%)
endogenous FVIII
activity as documented either by central laboratory testing at screening or in
historical
medical records from a clinical laboratory demonstrating <1% FVIII coagulant
activity
(FVIII:C) or a documented genotype known to produce severe hemophilia A.
- Previous treatment for hemophilia A (prophylaxis or on-demand) with any
recombinant
and/or plasma-derived FVIII, or cryoprecipitate for at least 150 EDs or 50 EDs
for
participants aged <6 years.
- Platelet count >=100 000 cells/µL at screening.
- Weight above or equal to 10 kg.

Arm B is only applicable for China

- History of hypersensitivity or anaphylaxis associated with any FVIII product.
- History of a positive inhibitor (to FVIII) test defined as >=0.6 BU/mL, or any
value greater than or equal to the lower sensitivity cut-off for laboratories
with cut-offs for inhibitor detection between 0.7 and 1.0 BU/mL, or clinical
signs or symptoms of decreased response to FVIII administrations. Family
history of inhibitors will not exclude the participant.
- Positive inhibitor test result, defined as >=0.6 BU/mL at Screening. Any
concurrent clinically significant liver disease.
- Serious active bacterial, fungal, or viral infection.
- Other known coagulation disorder(s) in addition to hemophilia A.
- Abnormal renal function or significant liver disease.