Recombinant Von Willebrand Factor in Subjects With Severe Von Willebrand Disease Undergoing Surgery

Inclusion Criteria:

- Diagnosis of severe von Willebrand disease (VWD) as listed below and elective surgical
procedure planned

1. Type 1 (Von Willebrand factor : Ristocetin cofactor activity (VWF:RCo) <20
IU/dL), or

2. Type 2A (as verified by multimer pattern), Type 2B (as diagnosed by genotype),
Type 2N (FVIII:C<10% and historically documented genetics), Type 2M, or

3. Type 3 (Von Willebrand factor antigen (VWF:Ag) ≤ 3 IU/dL)

- VWD with a history of requiring substitution therapy with von Willebrand factor (VWF)
concentrate to control bleeding

- If type 3 VWD (VWF Antigen /VWF:Ag ≤ 3 IU/dL), participant has a medical history of at
least 20 exposure days to VWF/FVIII coagulation factor concentrates (including
cryoprecipitate or fresh frozen plasma)

- If type 1 or type 2 VWD, participant has a medical history of 5 exposure days or a
past major surgery requiring VWF/FVIII coagulation factor concentrates (including
cryoprecipitate or fresh frozen plasma)

- Participant is at least 18 years of age

- If female of childbearing potential, participant presents with a negative pregnancy
test

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

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

Exclusion Criteria:

- 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)

- History or presence of a VWF inhibitor at screening

- History or presence of a factor VIII (FVIII) inhibitor with a titer ≥ 0.4 BU
(Nijmegen-modified Bethesda assay ) or ≥ 0.6 BU (by Bethesda assay)

- Known hypersensitivity to any of the components of the study drugs, such as to mouse
or hamster proteins

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

- Medical history of a thromboembolic event

- HIV positive with an absolute CD4 count < 200/mm3

- Platelet count < 100,000/mL

- Diagnosis of significant liver disease, as evidenced by, but not limited to, any of
the following: serum alanine aminotransferase (ALT) 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

- Diagnosis of renal disease, with a serum creatinine level ≥ 2 .5mg/dL

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

- Participant is pregnant or lactating at the time informed content is obtained

- Participant has participated in another clinical study involving an investigational
product (IP), other than 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. However,
eligible patients participating in the rVWF Prophylaxis Study (071301) may be
enrolled.

- Progressive fatal disease and/or life expectancy of less than 3 months

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

- Participant suffers from 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

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

- Participant is a member of the study team conducting this study or in a dependent
relationship with one of the study team members. Dependent relationships include close
relatives (ie, children, partner/spouse, siblings, parents) as well as employees.