RecruitingPhase 3NCT06998524

A Study to Assess the Efficacy and Safety of Emicizumab in Participants With Type 3 Von Willebrand Disease

A Phase III, Multicenter, Open-Label Study to Evaluate the Efficacy, Safety, Pharmacokinetics, and Pharmacodynamics of Emicizumab Prophylaxis in Patients With Type 3 Von Willebrand Disease


Sponsor

Hoffmann-La Roche

Enrollment

75 participants

Start Date

Jun 27, 2025

Study Type

INTERVENTIONAL

Conditions

Summary

This is a Phase III, multicenter, open-label clinical study designed to evaluate the efficacy, safety, pharmacokinetics, and pharmacodynamics of emicizumab prophylaxis in participants aged 1 month and above, who have been diagnosed with Type 3 von Willebrand disease (VWD). Participants on prior standard of care (SOC) on-demand therapy will be assessed via a randomized comparison (Arm A - emicizumab prophylaxis and Arm B - continuation of SOC on-demand therapy), while participants on prior SOC prophylactic therapy (Arm C - emicizumab prophylaxis) will be assessed via intra-participant analysis with data obtained from the preceding non-interventional study (NIS), WP45335 (NCT06883240).


Eligibility

Min Age: 1 Month

Inclusion Criteria10

  • Confirmed diagnosis of Type 3 von Willebrand disease (VWD), based on medical records
  • Preexisting medical record verifying the status of von Willebrand factor (VWF) inhibitor (positive or negative, including titer if available)
  • Adequate hematologic, hepatic, and renal function
  • For participants of childbearing potential: agreement to remain abstinent or adhere to the contraception requirements
  • Age ≥1 month at the time of signing Informed Consent/Assent Form
  • Documented previous use of on-demand therapy with intermittent (less than once a week) on-demand SOC therapy for VWD
  • Having ≥2 treated bleeds (except menstrual bleeds) with factor concentrate within 24 weeks prior to enrollment
  • Age ≥2 years at the time of signing Informed Consent/Assent Form
  • Documented and confirmed previous use of SOC prophylactic therapy for VWD (1-3 times weekly, as per prescribed dose) as described in the eligibility of Study WP45335
  • Have completed all study requirements as defined in the WP45335 protocol for at least 24 weeks

Exclusion Criteria7

  • Inherited or acquired bleeding disorder other than Congenital Type 3 VWD
  • History of gastrointestinal bleeding within 18 months prior to enrollment, or any previous diagnosis of angiodysplasia
  • History of intracranial hemorrhage
  • Previous or current treatment for thromboembolic disease or signs of thromboembolic disease
  • Other conditions (e.g., certain autoimmune diseases) that may increase risk of bleeding or thrombosis
  • History of clinically significant hypersensitivity associated with monoclonal antibody therapies or components of the emicizumab injection
  • Use of systemic immunomodulators (e.g., interferon) at enrollment or planned use during the study, with the exception of anti-retroviral therapy

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Interventions

DRUGEmicizumab

Participants will receive emicizumab 3 milligrams per kilogram (mg/kg) subcutaneous (SC) injections every week (QW) for the first 4 weeks as loading doses, followed by maintenance doses of emicizumab 3 mg/kg SC once every 2 weeks (Q2W). During the extension period, participants may remain on maintenance dose of emicizumab 3 mg/kg Q2W, or change their emicizumab maintenance regimen to 1.5 mg/kg once every week (QW) or 6 mg/kg once every 4 weeks (Q4W), if they prefer and if agreed by the investigators.

DRUGvon Willebrand Factor (VWF) Concentrates

Used according to local labeling or local treatment guidelines.

DRUGFactor VIII (FVIII) Concentrates

Used according to local labeling or local treatment guidelines.

DRUGvon Willebrand Factor (VWF) and Factor VIII (FVIII) Concentrates

Used according to local labeling or local treatment guidelines.

DRUGBypassing Agents

Used according to local labeling or local treatment guidelines.


Locations(27)

UC Davis

Sacramento, California, United States

University of Florida

Gainesville, Florida, United States

University of Minnesota Medical Center

Minneapolis, Minnesota, United States

Washington University School of Medicine

St Louis, Missouri, United States

UZ Leuven Gasthuisberg

Leuven, Belgium

The Hospital for Sick Children

Toronto, Ontario, Canada

McGill University Health Center

Montreal, Quebec, Canada

IPS SURA Industriales Medellín

Medellín, Colombia

Hopital Claude Huriez - CHU Lille

Lille, France

Groupe Hospitalier Necker Enfants Malades

Paris, France

Universitätsklinikum Bonn

Bonn, Germany

Gerinnungszentrum Rhein-Ruhr;Gerinnungsambulanz

Duisburg, Germany

Hämophiliezentrum Med. Klinik III/Institut für Transfusionsmedizin

Frankfurt/M., Germany

Universita' Degli Studi La Sapienza-Ist.Di Ematologia

Rome, Lazio, Italy

IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, Lombardy, Italy

AOU Careggi

Florence, Tuscany, Italy

Kurume University Hospital

Fukuoka, Japan

Nagoya University Hospital

Nagoya, Japan

Erasmus MC

Rotterdam, Netherlands

Instytut Hematologii i Transfuzjologii

Warsaw, Poland

Charlotte Maxeke Johannesburg Academic Hospital

Johannesburg, South Africa

Hospital Universitario la Paz

Madrid, Spain

Hospital Universitario Virgen del Rocio

Seville, Spain

Sahlgrenska Universitetssjukhuset

Gothenburg, Sweden

St Thomas' Hospital

London, United Kingdom

Great Ormond Street Hospital

London, United Kingdom

Manchester Royal Infirmary

Manchester, United Kingdom

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NCT06998524


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