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Phase 3 N=15 Treatment

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

Von Willebrand Disease

Enrolled (actual)
15
Serious AEs
13.3%
Results posted
Aug 2017
Primary outcome: Primary: Overall Hemostatic Efficacy as Assessed by the Investigator (Hemophilia Physician) — 11; 4; 7; 0 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
Recombinant von Willebrand Factor (rVWF) (Biological)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Baxalta now part of Shire
Primary completion
Jul 2016

Outcome Measures

OutcomeResultp-value
PRIMARY
Overall Hemostatic Efficacy as Assessed by the Investigator (Hemophilia Physician)
11; 4; 7; 0; 2; 1
SECONDARY
Intraoperative Actual Versus Predicted Blood Loss as Assessed by the Operating Surgeon
94.3; 0.0; 127.0; 145.0; 115.0; 42.5
SECONDARY
Intraoperative Actual Blood Loss Relative to Predicted Blood Loss
69.6; 0.0; 68.9; 145.0; 122.5; 50.0
SECONDARY
Intraoperative Actual Versus Predicted Blood Loss Score as Assessed by the Operating Surgeon
13; 4; 8; 1; 3; 1
SECONDARY
Intraoperative Hemostatic Efficacy Score as Assessed by the Operating Surgeon
13; 4; 8; 1; 3; 1
SECONDARY
Daily Intra- and Postoperative Weight-adjusted Dose of rVWF With or Without ADVATE
18.1; 23.5; 42.3; 28.6; 33.9; 31.5
SECONDARY
Occurrence of Adverse Events
12; 1; 0; 0; 0; 2
SECONDARY
Occurrence of Thrombotic Events
2; 1
SECONDARY
Occurrence of Severe Allergic Reactions (eg, Anaphylaxis)
0; 0
SECONDARY
Number of Participants Who Developed Inhibitory and Total Binding Antibodies to Von Willebrand Factor (VWF) and Inhibitory Antibodies to Factor VIII (FVIII)
0; 1; 0
SECONDARY
Number of Participants Who Developed Antibodies to Chinese Hamster Ovary (CHO) Proteins, Mouse Immunoglobulin G (IgG) or Recombinant Furin (rFurin)
SECONDARY
Pharmacokinetics: Area Under the Plasma Concentration Versus Time Curve From 0 to 72 Hours Post-infusion (AUC 0-72 h/Dose)
31.91; 57.08; 63.91; 54.61; 67.49
SECONDARY
Pharmacokinetics: Area Under the Plasma Concentration Versus Time Curve From Time 0 to Infinity (AUC 0-∞ /Dose)
34.43; 68.87; 71.82; 61.90; 75.00
SECONDARY
Pharmacokinetics: Mean Residence Time (MRT)
22.69; 37.92; 29.35; 29.75
SECONDARY
Pharmacokinetics: Clearance (CL)
0.02904; 0.01452; 0.01392; 0.01616
SECONDARY
Pharmacokinetics: Incremental Recovery (IR)
1.961; 1.991; 2.780; 2.635
SECONDARY
Pharmacokinetics: Elimination Phase Half-life (T1/2)
16.52; 26.88; 21.07; 22.19
SECONDARY
Pharmacokinetics: Volume of Distribution at Steady State (Vss)
0.6591; 0.5506; 0.4086; 0.4806

Summary

The purpose of the study is to assess the efficacy and safety of recombinant von Willebrand factor (rVWF) with or without ADVATE in major and minor elective surgical procedures in adult patients with hereditary severe von Willebrand disease (VWD).

Eligibility Criteria

Inclusion Criteria

  • Diagnosis of severe von Willebrand disease (VWD) as listed below and elective surgical procedure planned
  • Type 1 (Von Willebrand factor : Ristocetin cofactor activity (VWF:RCo) 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.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02283268). 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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