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Phase 3 N=37 Treatment

Pharmacokinetics, Safety and Efficacy of Recombinant Von Willebrand Factor (rVWF) in the Treatment of Bleeding Episodes in Von Willebrand Disease (VWD)

Von Willebrand Disease

Enrolled (actual)
37
Serious AEs
18.9%
Results posted
Apr 2016
Primary outcome: Primary: Percentage of Participants With Treatment Success for Treated Bleeding Episodes — 100.0 Percent of participants

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
Recombinant von Willebrand factor (rVWF) (Biological); Placebo (Drug); Recombinant factor VIIII (rFVIII) (Biological)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Baxalta now part of Shire
Primary completion
Feb 2014

Outcome Measures

OutcomeResultp-value
PRIMARY
Percentage of Participants With Treatment Success for Treated Bleeding Episodes
100.0
SECONDARY
Percentage of Treated Bleeding Episodes With an Efficacy Rating of "Excellent" or "Good"
100.0
SECONDARY
Percentage of Treated Bleeding Episodes With an Efficacy Rating of "Excellent" or "Good", Excluding Gastrointestinal Bleeds
100.0
SECONDARY
Number of Infusions of rVWF:rFVIII and/or rVWF Per Bleeding Episode
1.0
SECONDARY
Number of Units of rVWF:rFVIII and/or rVWF Per Bleeding Episode
48.2
SECONDARY
Percentage of Participants Who Develop Inhibitory Antibodies to FVIII
0; 0; 0
SECONDARY
Percentage of Participants Who Develop Inhibitory Antibodies to VWF
0; 0; 0
SECONDARY
Percentage of Participants Who Develop Binding Antibodies to VWF
0; 0; 0
SECONDARY
Percentage of Participants Who Develop Binding Antibodies to CHO
0; 0; 0
SECONDARY
Percentage of Participants Who Develop Binding Antibodies to rFurin
0; 0; 0
SECONDARY
Percentage of Participants Who Develop Binding Antibodies to Mouse Immunoglobulin
2.8; 0; 0
SECONDARY
Percentage of Participants Who Had an Occurrence of Thrombotic Events
SECONDARY
Number of Adverse Events Related to Study Product Including Clinically Significant Changes in Laboratory Parameters and Vital Signs
1; 1; 1; 1; 1; 1
SECONDARY
Number of Participants With Adverse Events Related to Study Product Including Clinically Significant Changes in Laboratory Parameters and Vital Signs
1; 1; 1; 1; 1; 1
SECONDARY
Number of Adverse Events by Infusion Related to Study Product Including Clinically Significant Changes in Laboratory Parameters and Vital Signs
1; 1; 1; 1; 1; 1
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:RCo
32.4; 32.7
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:RCo
31.6; 31.3
SECONDARY
PK50 - Mean Residence Time of VWF:RCo
25.2; 26.7
SECONDARY
PK50 - Clearance of VWF:RCo
0.031; 0.031
SECONDARY
PK50 - Incremental Recovery of VWF:RCo
1.8; 1.8
SECONDARY
PK50 - Elimination Phase Half-Life of VWF:Co
16.6; 19.4
SECONDARY
PK50 - Volume of Distribution at Steady State of VWF:RCo
0.70; 0.83
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:Ag
67.8; 67.1
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:Ag
62.1; 62.2
SECONDARY
PK50 - Mean Residence Time of VWF:Ag
32.1; 34.3
SECONDARY
PK50 - Clearance of VWF:Ag
0.015; 0.015
SECONDARY
PK50 - Incremental Recovery of VWF:Ag
2.3; 2.2
SECONDARY
PK50 - Elimination Phase Half-Life of VWF:Ag
21.8; 25.2
SECONDARY
PK50 - Volume of Distribution at Steady State of VWF:Ag
0.50; 0.49
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:CB
80.1; 81.3
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:CB
78.7; 75.1
SECONDARY
PK50 - Mean Residence Time of VWF:CB
27.5; 26.1
SECONDARY
PK50 - Clearance of VWF:CB
0.012; 0.012
SECONDARY
PK50 - Incremental Recovery of VWF:CB
3.4; 3.2
SECONDARY
PK50 - Elimination Phase Half-Life of VWF:CB
19.3; 18.3
SECONDARY
PK50 - Volume of Distribution at Steady State of VWF:CB
0.35; 0.36
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of FVIII:C
145.4; 113.0
SECONDARY
PK50 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of FVIII:C
127.8; 101.8
SECONDARY
PK50 - Mean Residence Time of FVIII:C
44.0
SECONDARY
PK50 - Clearance of FVIII:C
0.007
SECONDARY
PK50 - Incremental Recovery of FVIII:C
2.3
SECONDARY
PK50 - Elimination Phase Half-Life of FVIII:C
24.8
SECONDARY
PK50 - Volume of Distribution at Steady State of FVIII:C
0.32
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:RCo
36.9; 38.9
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:RCo
35.6; 37.9
SECONDARY
PK80 - Mean Residence Time of VWF:RCo
26.4; 26.4
SECONDARY
PK80 - Clearance of VWF:RCo
0.027; 0.026
SECONDARY
PK80 - Incremental Recovery of VWF:RCo
1.8; 1.8
SECONDARY
PK80 - Elimination Phase Half-Life of VWF:Co
18.4; 19.8
SECONDARY
PK80 - Volume of Distribution at Steady State of VWF:RCo
0.78; 0.75
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:Ag
66.6; 86.9
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:Ag
61.3; 77.4
SECONDARY
PK80 - Mean Residence Time of VWF:Ag
38.4; 36.9
SECONDARY
PK80 - Clearance of VWF:Ag
0.015; 0.012
SECONDARY
PK80 - Incremental Recovery of VWF:Ag
2.2; 2.4
SECONDARY
PK80 - Elimination Phase Half-Life of VWF:Ag
27.5; 24.8
SECONDARY
PK80 - Volume of Distribution at Steady State of VWF:Ag
0.55; 0.50
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of VWF:CB
73.9; 90.8
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of VWF:CB
71.9; 88.1
SECONDARY
PK80 - Mean Residence Time of VWF:CB
30.9; 28.7
SECONDARY
PK80 - Clearance of VWF:CB
0.014; 0.011
SECONDARY
PK80 - Incremental Recovery of VWF:CB
3.1; 3.7
SECONDARY
PK80 - Elimination Phase Half-Life of VWF:CB
18.8; 20.9
SECONDARY
PK80 - Volume of Distribution at Steady State of VWF:CB
0.39; 0.36
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-∞/Dose) of FVIII:C
96.8; 94.8
SECONDARY
PK80 - Area Under the Plasma Concentration/Time Curve From Time 0 to 96 Hours (AUC0-96h/Dose) of FVIII:C
81.7; 71.8
SECONDARY
PK80- Ratio of Intra-participant PK of VWF:RCo, VWF:Ag and VWF:CB at Baseline and After 6 Months
0.9587; 1.0914; 1.0666

Summary

The purpose of this Phase 3 study is to assess the pharmacokinetics of rVWF:rFVIII and rVWF, and to assess the safety and efficacy of rVWF:rFVIII and rVWF in the treatment of bleeding events in subjects with severe hereditary von Willebrand disease (VWD).

Eligibility Criteria

Inclusion Criteria

  • Participant has been diagnosed with:
  • Type 1 (Von Willebrand factor: Ristocetin cofactor activity (VWF:RCo) 1.4).
  • Participant has a documented history of a VWF:RCo half-life of <6 hours.
  • Participant has a history or presence of a VWF inhibitor at screening.
  • Participant has a history or presence of a factor VIII (FVIII) inhibitor with a titer ≥0.4 BU (by Nijmegen assay) or ≥ 0.6 BU (by Bethesda assay).
  • Participant has a known hypersensitivity to any of the components of the study drugs, such as mouse or hamster proteins.
  • Participant has a medical history of immunological disorders, excluding seasonal allergic rhinitis/conjunctivitis, mild asthma, food allergies or animal allergies.
  • Participant has a medical history of a thromboembolic event.
  • Participant is HIV positive with an absolute CD4 count <200/mm3.
  • Participant has been diagnosed with cardiovascular disease (New York Heart Association [NYHA] classes 1-4.
  • Participant has an acute illness (eg, influenza, flu-like syndrome, allergic rhinitis/conjunctivitis, non-seasonal asthma) at screening.
  • 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; hypoalbuminemia; portal vein hypertension (eg, presence of otherwise unexplained splenomegaly, history of esophageal varices).
  • Participant has been diagnosed with renal disease, with a serum creatinine level ≥2 mg/dL.
  • In the judgment of the investigator, the participant has another clinically significant concomitant disease (eg, uncontrolled hypertension) that may pose additional risks for the participant.
  • Participant has been treated with an immunomodulatory drug, excluding topical treatment (eg, ointments, nasal sprays), within 30 days prior to enrollment
  • Participant is pregnant or lactating at the time of enrollment.
  • Participant has participated in another clinical study involving an IP or investigational device within 30 days prior to enrollment or is scheduled to participate in another clinical study involving an investigational product or investigational device during the course of this study.
  • Participant has a history of drug or alcohol abuse within the 2 years prior to enrollment.
  • Participant has a 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
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01410227). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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