A Trial That Evaluates Disease Characteristics in Hemophilia B Adult Male Participants Receiving Prophylaxis With Standard of Care Factor IX Protein (FIX) Replacement Therapy

NCT ID: NCT05568459

Last Updated: 2025-12-19

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Total Enrollment

11 participants

Study Classification

OBSERVATIONAL

Study Start Date

2024-01-17

Study Completion Date

2026-01-21

Brief Summary

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This study is focused on males who have Hemophilia B and who need regular preventive treatment with factor IX protein (FIX) replacement therapy to prevent and also to control their bleeding events. The aim of the study is to gather at least 6 months of information on bleeding events for each individual participant while they continue to use their usual FIX replacement therapy. There is no experimental treatment being tested in this study. The study is informational, and part of a larger program to understand and treat Hemophilia B with a potential experimental new therapy in the future. There is no obligation to agree to taking part in this future study.

The study is looking to answer several other research questions to help understand each participant's individual disease characteristics, including:

* How often to use FIX replacement therapy, both on a regular basis (prophylaxis) and as needed to treat bleeding events
* Measurement of FIX activity (factor IX is a clotting factor) by different laboratories using different types of tests in Hemophilia B participants
* Possible complications from the FIX replacement therapy the patient receives (usual standard of care will continue to be used)
* How quality of life is affected by Hemophilia B
* How joint health is affected by Hemophilia B
* How often the participant visits the emergency room, urgent care center, physician's office, hospital, or has a telemedicine visit as a result of bleeding events
* Whether the body makes antibodies (a protein produced by the body's immune system) against the FIX replacement therapy you receive, which could make the drug less effective or could lead to side effects

Detailed Description

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Conditions

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Hemophilia B

Keywords

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FIX replacement therapy

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Cohort 1

Male participants with hemophilia B on current FIX Replacement Therapy prophylaxis or a documented genotype known to produce severe hemophilia B

Non-Interventional

Intervention Type OTHER

No study treatment will be administered in this study.

Interventions

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Non-Interventional

No study treatment will be administered in this study.

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

1. Previous experience with FIX therapy (≥50 documented exposure days to a FIX protein product such as recombinant, plasma-derived or extended half-life FIX product) with a current stable prophylaxis regimen for \>2 months prior to enrollment and intention to use FIX replacement therapy for the duration of the study
2. No known hypersensitivity to FIX replacement product
3. Willing to be contacted about a potential future clustered regularly interspaced short palindromic repeats (CRISPR)-based Factor 9 (F9) gene insertion clinical trial in which they may have the opportunity to screen for enrollment

Exclusion Criteria

1. History of any coagulation disorder; requires anticoagulant therapy
2. Lack of adherence with documentation of bleeds and/or prophylaxis replacement therapy administration in the opinion of the investigator, based on medical history
3. History of FIX inhibitor (clinical or laboratory-based assessment) on 2 or more occasions, as defined in the protocol
4. Bethesda inhibitor titer greater than the upper limit of normal (ULN) at screening
5. Any detectable pre-existing antibodies to the Adeno-associated virus serotype 8 (AAV8) capsid; as measured by an assay at prescreening, as defined in the protocol
6. Is positive for hepatitis B or C at screening, as defined in protocol
7. If any of the following pre-existing diagnoses are documented:

* Cholestatic liver disease
* Liver cirrhosis
* Portal hypertension; or
* Splenomegaly; or
* Hepatic encephalopathy
8. History of arterial or venous thrombo-embolic events, as defined in the protocol
9. History of clinically significant cardiovascular, respiratory, hepatic, renal (including nephrotic syndrome), gastrointestinal (including protein-losing enteropathy), endocrine, hematological (including thrombophilia), psychiatric, or neurological disease, as assessed by the investigator that may confound the results of the study or poses an additional risk to the participant by study participation
10. Previously received of any AAV-gene based therapy with a marketed gene therapy or in a clinical trial or intent to receive approved or investigational AAV-gene based therapy during the study period

NOTE: Other Inclusion/Exclusion Protocol Defined Criteria Apply
Minimum Eligible Age

16 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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Regeneron Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Clinical Trial Management

Role: STUDY_DIRECTOR

Regeneron Pharmaceuticals

Locations

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University of Colorado Hemophilia and Thrombosis Center

Aurora, Colorado, United States

Site Status

Yale HTC

New Haven, Connecticut, United States

Site Status

Georgetown University Medical Center

Washington D.C., District of Columbia, United States

Site Status

University of Florida

Gainesville, Florida, United States

Site Status

Rush University Medical Center

Chicago, Illinois, United States

Site Status

Indiana Hemophilia and Thrombosis Center

Indianapolis, Indiana, United States

Site Status

University of Michigan

Ann Arbor, Michigan, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

University of Texas Health Science Center at Houston

Houston, Texas, United States

Site Status

McMaster University Medical Centre - Hamilton Health Sciences

Hamilton, Ontario, Canada

Site Status

Mcgill University Health Center (MUHC)

Montreal, Quebec, Canada

Site Status

University Hospital of Regensburg

Regensburg, Bavaria, Germany

Site Status

Klinikum der Johann Wolfgang Goethe-Universitaet Frankfurt

Frankfurt, , Germany

Site Status

University Hospital Hamburg Eppendorf

Hamburg, , Germany

Site Status

Southampton General Hospital

Southampton, Hampshire, United Kingdom

Site Status

Barts Health NHS Trust, Royal London Hospital

London, , United Kingdom

Site Status

St. Thomas' Hospital

London, , United Kingdom

Site Status

Countries

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United States Canada Germany United Kingdom

Other Identifiers

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R0000-HEMB-2187

Identifier Type: -

Identifier Source: org_study_id