BAX 111 rVWF in Pediatrics

Inclusion Criteria:

1. Diagnosis of severe von Willebrand disease (VWD) (defined as von Willebrand factor:
ristocetin cofactor [VWF:RCo] <20%):

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

2. Type 2A (VWF:RCo <20 IU/dL), Type 2B (as diagnosed by genotype), Type 2N (Factor
VIII coagulation activity [FVIII:C] <10% and historically documented genetics),
Type 2M; or

3. Type 3 (VWF:Ag ≤3 IU/dL).

2. Age 0 to <18 years at the time of Screening

3. The participant has provided assent (if appropriate) and legally authorized
representative(s) has provided informed consent

4. If female of childbearing potential, participant presents with a negative serum
pregnancy test

5. If applicable, participant agrees to employ adequate birth control measures for the
duration of the study

6. The participant and/or the legal representative is willing and able to comply with the
requirements of the protocol

Additional inclusion criteria for previously treated participants and participants
undergoing surgery are as follows:

1. Unable to tolerate or are inadequately responsive to deamino-delta-D-arginine
vasopressin

2. The participant has had a minimum of 1 documented bleed requiring VWF coagulation
factor replacement therapy during the previous 12 months prior to enrollment and
overall historically 3 or more exposure days (EDs) to plasma-derived VWF

Additional inclusion criterion for previously untreated participants are as follows:

1. The participant has not received prior VWF coagulation factor replacement therapy

Exclusion Criteria:

1. Diagnosis of pseudo-VWD or another hereditary or acquired coagulation disorder (eg,
qualitative and quantitative platelet disorders or elevated prothrombin time
[PT]/international normalized ratio [INR] >1.4)

2. History or presence of a VWF inhibitor at Screening

3. History or presence of a Factor VIII (FVIII) inhibitor with a titer ≥0.4 Bethesda
units (BU) (by Nijmegen assay) or ≥0.6 BU (by Bethesda assay)

4. Documented history of a VWF:RCo half-life <6 hours

5. Known hypersensitivity to any of the components of the study drug, such as mouse or
hamster proteins

6. Medical history of immunological disorders, excluding seasonal allergic
rhinitis/conjunctivitis/asthma, food allergies, or animal allergies

7. Medical history of a thromboembolic event

8. Human immunodeficiency virus (HIV) positive, with an absolute CD4 count <200/mm^3

9. In the judgment of the Investigator, the participant has another clinically
significant concomitant disease (eg, uncontrolled hypertension, cancer) that may pose
additional risks for the participant

10. Diagnosis of significant liver disease, as evidenced by, but not limited to, any of
the following: serum alanine aminotransferase (ALT) of 5 times the upper limit of
normal; hypoalbuminemia; portal vein hypertension (eg, presence of otherwise
unexplained splenomegaly, history of esophageal varices) or liver cirrhosis classified
as Child B or C

11. Diagnosis of renal disease, with a serum creatinine level ≥2.5 mg/dL

12. Immunomodulatory drug treatment other than anti-retroviral chemotherapy (eg,
α-interferon, or corticosteroid agents at a dose equivalent to hydrocortisone greater
than 10 mg/day (excluding topical treatment [eg, ointments, nasal sprays]), within 30
days prior to signing the informed consent (or assent, if appropriate)

13. If female, participant is pregnant or lactating at the time informed consent (or
assent, if appropriate) is obtained

14. Participant has participated in another clinical study involving an investigational
product (IP), other than recombinant von Willebrand Factor (rVWF) with or without
ADVATE, 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