rVWF IN PROPHYLAXIS

Inclusion Criteria:

1. Participant has a documented diagnosis of severe von Willebrand disease (VWD)
(baseline Von Willebrand factor: Ristocetin cofactor activity (VWF:RCo) <20 IU/dL)
with a history of requiring substitution therapy with von Willebrand factor
concentrate to control bleeding

1. Type 1 (VWF:RCo <20 IU/dL) or,

2. Type 2A (as verified by multimer pattern), Type 2B (as diagnosed by genotype),
Type 2M or,

3. Type 3 (VWF:Ag ≤3 IU/dL).

2. Diagnosis is confirmed by genetic testing and multimer analysis, documented in patient
history or at screening.

3. Participant currently receiving on-demand treatment for whom prophylactic treatment is
recommended according to standard of care at the center.

4. Has ≥3 documented spontaneous bleeds requiring von Willebrand factor (VWF) treatment
during the past 12 months

5. Availability of records to reliably evaluate type, frequency and treatment of bleeding
episodes during 12 months of on-demand treatment prior to enrollment.

6. Participant is ≥18 years old at the time of screening and has a body mass index ≥15
but <40 kg/m^2.

7. If female of childbearing potential, participant presents with a negative blood/urine
pregnancy test at screening and agrees to employ adequate birth control measures for
the duration of the study.

8. Participant is willing and able to comply with the requirements of the protocol.

Exclusion Criteria:

1. Participant has been diagnosed with Type 2N von Willebrand disease (VWD), pseudo VWD,
or another hereditary or acquired coagulation disorder other than VWD (eg qualitative
and quantitative platelet disorders or elevated prothrombin time (PT)/ international
normalized ratio (INR) >1.4).

2. Participant has received prophylaxis treatment in the 12 months prior to screening
(including those who received treatment once a month for menorrhagia but were not
treated for any other bleeds).

3. Participant is currently receiving prophylaxis treatment.

4. Participant has a history or presence of a VWF inhibitor at screening.

5. Participant has a history or presence of a Factor VIII (FVIII) inhibitor with a titer
≥0.4 BU (by Nijmegen modified Bethesda assay) or ≥0.6 Bethesda Unit (BU) (by Bethesda
assay).

6. Participant has a known hypersensitivity to any of the components of the study drugs,
such as to mouse or hamster proteins.

7. Participant has a medical history of immunological disorders, excluding seasonal
allergic rhinitis/conjunctivitis, mild asthma, food allergies or animal allergies.

8. Participant has a medical history of a thromboembolic event.

9. Participant is human immunodeficiency virus (HIV) positive with an absolute Helper T
cell (CD4) count <200/mm^3.

10. Participant has been diagnosed with significant liver disease as evidenced by any of
the following: serum alanine aminotransferase (ALT) 5 times the upper limit of normal
(ULN); hypoalbuminemia; portal vein hypertension (e.g., presence of otherwise
unexplained splenomegaly, history of esophageal varices).

11. Participant has been diagnosed with renal disease, with a serum creatinine level ≥2.5
mg/dL.

12. Participant has a platelet count <100,000/mL at screening.

13. Participant has been treated with an immunomodulatory drug, excluding topical
treatment (e.g., ointments, nasal sprays), within 30 days prior to signing the
informed consent.

14. Participant is pregnant or lactating at the time of enrollment.

15. Participant has cervical or uterine conditions causing menorrhagia or metrorrhagia
(including infection, dysplasia).

16. Participant has participated in another clinical study involving another
Investigational Product (IP) or investigational device within 30 days prior to
enrollment or is scheduled to participate in another clinical study involving an IP or
investigational device during the course of this study.

17. Participant has a progressive fatal disease and/or life expectancy of less than 15
months.

18. Participant is identified by the investigator as being unable or unwilling to
cooperate with study procedures.

19. The subject has a mental condition rendering him/her unable to understand the nature,
scope and possible consequences of the study and/or evidence of an uncooperative
attitude.

20. Participant is in prison or compulsory detention by regulatory and/or juridical order.

21. Participant is member of the study team or in a dependent relationship with one of the
study team members which includes close relatives (i.e., children, partner/spouse,
siblings and parents) as well as employees.