A Study of Recombinant Von Willebrand Factor (rVWF) in Pediatric and Adult Participants With Severe Von Willebrand Disease (VWD)

Inclusion Criteria:

The participant will not be considered eligible for the study without meeting all of the criteria below.

Participants who have completed Study 071301 or Study 071102 (or participants who have completed the surgery arm treatment in Study 071102 and want to continue to receive on-demand (OD) treatment) and are willing to immediately transition into this study, must meet the following 2 criteria to be eligible for this study:

• If female of childbearing potential, has a negative blood/urine pregnancy test atscreening and agrees to employ highly effective birth control measures for theduration of the study.

• Participant and/or legally authorized representative is willing and able to complywith the requirements of the protocol.

New participants (Cohort 4) who meet the above 2 and ALL the following additional criteria are eligible for this study:

• Participant has a documented diagnosis of severe von Willebrand disease (VWD) (baseline von Willebrand factor: Ristocetin cofactor (VWF:RCo) <20 InternationalUnits per deciliter [IU/dL]) with a history of requiring substitution therapy withvon Willebrand factor (vWF) concentrate to control bleeding:

• Type 1 (VWF:RCo <20 IU/dL) or,

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

• Type 3 (Von Willebrand factor antigen (VWF:Ag) less than or equal to (<=) 3 IU/dL).

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

• Participant has been receiving OD therapy with VWF products for at least 12 months,and prophylactic treatment is recommended by the investigator.

• Participant has greater than or equal to (>=) 3 documented spontaneous bleeds (notincluding menorrhagia) requiring VWF treatment during the past 12 months.

• Participant has available records that reliably evaluate type, frequency, andtreatment of bleeding episodes for at least 12 months preceding enrollment; up to 24months of retrospective data should be collected if available.

• Participant is >=12 years old at the time of screening and has a body mass index >=15 but <40 kilogram per meter square (kg/m^2).

Exclusion Criteria:

The participant will be excluded from the study if any of the following exclusion criteria are met.

• The participant has been diagnosed with Type 2N VWD, pseudo VWD, or anotherhereditary or acquired coagulation disorder other than VWD (eg, qualitative andquantitative platelet disorders or elevated prothrombin time (PT)/internationalnormalized ratio [INR] >1.4).

• The participant has a history or presence of a VWF inhibitor at screening.

• The participant has a history or presence of a Factor VIII (FVIII) inhibitor with atiter >=0.4 Bethesda units (BU) (by Nijmegen modified Bethesda assay) or >=0.6 BU (by Bethesda assay).

• The participant has a known hypersensitivity to any of the components of the studydrugs, such as mouse or hamster proteins.

• The participant has a medical history of immunological disorders, excluding seasonalallergic rhinitis/conjunctivitis, mild asthma, food allergies, or animal allergies.

• The participant has a medical history of a thromboembolic event.

• The participant is human immunodeficiency virus (HIV) positive with an absoluteHelper T cell (CD4) count <200/cubic millimeters (mm^3).

• The participant has been diagnosed with significant liver disease per investigator'smedical assessment of the participant's current condition or medical history or asevidenced by, but not limited to any of the following: serum alanineaminotransferase (ALT) greater than 5 times the upper limit of normal;hypoalbuminemia; portal vein hypertension (eg, presence of otherwise unexplainedsplenomegaly, history of esophageal varices) or liver cirrhosis classified asChild-Pugh class B or C.

• The participant has been diagnosed with renal disease, with a serum creatinine (CR)level >=2.5 milligrams per deciliter (mg/dL).

• The participant has a platelet count <100,000/milliliter (mL) at screening.

• The participant has been treated with an immunomodulatory drug, excluding topicaltreatment (eg, ointments, nasal sprays), within 30 days prior to signing theinformed consent (or assent, if appropriate).

• The participant is pregnant or lactating at the time of enrollment.

• The participant has cervical or uterine conditions causing menorrhagia ormetrorrhagia (including infection, dysplasia).

• The participant has participated in another clinical study involving anotherinvestigational product (IP) or investigational device within 30 days prior toenrollment or is scheduled to participate in another clinical study involving an IPor investigational device during the course of this study.

• The participant has a progressive fatal disease and/or life expectancy of less than 15 months.

• For new OD participants, the participant is scheduled for a surgical intervention.

• The participant is identified by the investigator as being unable or unwilling tocooperate with study procedures.

• The participant has a mental condition rendering him/her unable to understand thenature, scope and possible consequences of the study and/or evidence of anuncooperative attitude.

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

Delay criteria Only for Cohort 4, if the participant presents with an acute bleeding episodes or acute illness (eg, influenza, flu-like syndrome, allergic rhinitis/conjunctivitis, and non-seasonal asthma) the screening visit will be postponed until the participant has recovered. For all other participants, end of study (EOS) visit for 071102 or 071301 will be completed per protocol and the completed EOS in Study 071102 or 071301 will also serve as the screening visit for this continuation study (SHP677-304).