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A Study Evaluating the Efficacy and Safety of Mitapivat in Participants With Transfusion-Dependent Alpha- or Beta-Thalassemia (α- or β-TDT)

Phase 3
Active, not recruiting
Conditions
Transfusion-dependent Alpha-Thalassemia
Transfusion-dependent Beta-Thalassemia
Interventions
Registration Number
NCT04770779
Lead Sponsor
Agios Pharmaceuticals, Inc.
Brief Summary

The primary purpose of this study is to compare the effect of mitapivat versus placebo on transfusion burden in participants with transfusion-dependent alpha- or beta-thalassemia (TDT).

Detailed Description

The mitapivat group will include approximately 160 participants. The placebo group will include approximately 80 participants.

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
258
Inclusion Criteria
  • Documented diagnosis of thalassemia (β-thalassemia with or without α-globin gene mutations, hemoglobin E (HbE)/β-thalassemia, or α-thalassemia/hemoglobin H (HbH) disease) based on deoxyribonucleic acid (DNA) analysis;
  • Considered transfusion-dependent, defined as 6 to 20 RBC units transfused and ≤6-week transfusion-free period during the 24-week period before randomization;
  • If taking hydroxyurea, the hydroxyurea dose must be stable for ≥16 weeks before randomization;
  • Women of childbearing potential (WOCBP) must be abstinent of sexual activities that may induce pregnancy as part of their usual lifestyle or agree to use two forms of contraception, one of which must be considered highly effective, from the time of providing informed consent, throughout the study, and for 28 days after the last dose of study drug. The second form of contraception can be an acceptable barrier method;
  • Written informed consent before any study-related procedures are conducted and willing to comply with all study procedures for the duration of the study.
Exclusion Criteria
  • Pregnant, breastfeeding, or parturient;

  • Documented history of homozygous or heterozygous sickle hemoglobin (Hb S) or hemoglobin C (Hb C);

  • Prior exposure to gene therapy or prior bone marrow or stem cell transplantation;

  • Currently receiving treatment with luspatercept; the last dose must have been administered ≥36 weeks before randomization;

  • Currently receiving treatment with hematopoietic stimulating agents; the last dose must have been administered ≥36 weeks before randomization;

  • History of malignancy (active or treated) ≤5 years before providing informed consent, except for nonmelanomatous skin cancer in situ, cervical carcinoma in situ, or breast carcinoma in situ;

  • History of active and/or uncontrolled cardiac or pulmonary disease ≤6 months before providing informed consent;

  • Hepatobiliary disorders;

  • Estimated glomerular filtration rate <45 milliliters per minute (mL/min)/1.73 meter (m)^2 by Chronic Kidney Disease Epidemiology Collaboration creatinine equation;

  • Nonfasting triglycerides >440 milligrams per deciliter (mg/dL) (5 millimoles per liter [mmol/L]);

  • Active infection requiring systemic antimicrobial therapy at the time of providing informed consent;

  • Positive test for hepatitis C virus antibody (HCVAb) with evidence of active HCV infection, or positive test for hepatitis B surface antigen (HBsAg);

  • Positive test for human immunodeficiency virus (HIV)-1 antibody (Ab) or HIV-2 Ab;

  • History of major surgery (including splenectomy) ≤6 months before providing informed consent and/or a major surgical procedure planned during the study;

  • Current enrollment or past participation (≤12 weeks before administration of the first dose of study drug or a timeframe equivalent to 5 half-lives of the investigational study drug, whichever is longer) in any other clinical study involving an investigational treatment or device;

  • Receiving strong CYP3A4/5 inhibitors that have not been stopped for ≥5 days or a timeframe equivalent to 5 half-lives (whichever is longer); or strong CYP3A4 inducers that have not been stopped for ≥4 weeks or a timeframe equivalent to 5 half-lives (whichever is longer), before randomization;

  • Receiving anabolic steroids that have not been stopped for at least 4 weeks before randomization. Testosterone replacement therapy to treat hypogonadism is allowed. The testosterone dose and preparation must be stable for ≥12 weeks before randomization;

  • Known allergy, or other contraindication, to mitapivat or its excipients (microcrystalline cellulose, croscarmellose sodium, sodium stearyl fumarate, mannitol, and magnesium stearate, Opadry® II Blue [hypromellose, titanium dioxide, lactose monohydrate, triacetin, and FD&C Blue #2]);

  • Any medical, hematological, psychological, or behavioral condition(s) or prior or current therapy that, in the opinion of the Investigator, may confer an unacceptable risk to participating in the study and/or could confound the interpretation of the study data. Also excluded are:

    • Participants who are institutionalized by regulatory or court order
    • Participants with any condition(s) that could create undue influence (including but not limited to incarceration, involuntary psychiatric confinement, and financial or familial affiliation with the Investigator or Sponsor).

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlacebo Matching MitapivatDouble-Blind Period: Participants will receive placebo matching mitapivat orally, BID for 48 weeks. Open-label Extension Period: Participants who do not discontinue study drug may choose to receive mitapivat for up to an additional 5 years after the Double-blind Period.
MitapivatMitapivatDouble-Blind Period: Participants will receive mitapivat 100 milligrams (mg) orally, twice daily (BID) for 48 weeks. Open-label Extension Period: Participants who do not discontinue study drug may choose to continue to receive mitapivat for up to an additional 5 years after the Double-blind Period.
PlaceboMitapivatDouble-Blind Period: Participants will receive placebo matching mitapivat orally, BID for 48 weeks. Open-label Extension Period: Participants who do not discontinue study drug may choose to receive mitapivat for up to an additional 5 years after the Double-blind Period.
Primary Outcome Measures
NameTimeMethod
Percentage of Participants With Transfusion Reduction Response (TRR)Baseline up to Week 48

TRR is defined as ≥50% reduction in transfused red blood cells (RBC) units with a reduction of ≥2 units of transfused RBCs in any consecutive 12-week period through Week 48 compared with baseline.

Secondary Outcome Measures
NameTimeMethod
Percentage of Participants With ≥50% Reduction in Transfused RBC Units in Any Consecutive 24-week Period Through Week 48 Compared With BaselineBaseline up to Week 48
Percentage of Participants With ≥50% Reduction in Transfused RBC Units From Week 13 Through Week 48 Compared With BaselineBaseline, Week 13 up to Week 48
Change From Baseline in Total Iron Binding Capacity Through Week 48Baseline, Week 48
Percentage of Participants With Adverse Events (AEs) and Serious Adverse Events (SAEs)Up to Week 317
Percentage of Participants with Adverse Events (AEs) Considered by the Investigator to be Related to Study DrugUp to Week 317
Percentage of Participants With ≥33% Reduction in Transfused RBC Units From Week 13 Through Week 48 Compared With BaselineBaseline, Week 13 up to Week 48
Change From Baseline in Transfused RBC Units From Week 13 Through Week 48Baseline, Week 13 up to Week 48
Percentage of Participants With Transfusion-IndependenceUp to Week 48

Transfusion-independence is defined as transfusion-free for ≥8 consecutive weeks through Week 48.

Change From Baseline in Serum Ferritin Through Week 48Baseline, Week 48
Change From Baseline in Transferrin Saturation Through Week 48Baseline, Week 48
Time of Maximum Plasma Concentration (Tmax) of MitapivatPre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36
Blood Concentration of 2,3 - diphosphoglycerate (2,3-DPG)Pre-dose Day 1; pre-dose Week 12; pre-dose Week 24; pre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36
Change From Baseline in Iron Through Week 48Baseline, Week 48
Percentage of Participants with Serious Adverse Events (SAEs) Considered by the Investigator to be Related to Study DrugUp to Week 317
Plasma or Blood Concentrations Over Time for MitapivatPre-dose Week 12; pre-dose Week 24; pre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36
Area Under the Concentration-time Curve From Time Zero to the Last Measurable Concentration (AUClast) of MitapivatPre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36
Maximum Plasma Concentration (Cmax) of MitapivatPre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36
Percentage of Participants with Adverse Events (AEs) and Serious Adverse Events (SAEs), Graded by SeverityUp to Week 317

AEs will be graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE; Version 4.03) from Grades 1 to 4 where Grade 1 is mild and Grade 4 is life-threatening.

Blood Concentration of Adenosine Triphosphate (ATP)Pre-dose Day 1; pre-dose Week 12; pre-dose Week 24; pre-dose, 0.5, 1, 3, 5, 7 hours post-dose Week 36

Trial Locations

Locations (78)

Penn Medicine - University of Pennsylvania Health System

🇺🇸

Philadelphia, Pennsylvania, United States

Phoenix Children's Hospital

🇺🇸

Phoenix, Arizona, United States

Ospedale Pediatrico Microcitemico

🇮🇹

Cagliari, Italy

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Children's Hospital Oakland

🇺🇸

Oakland, California, United States

Stanford Medicine

🇺🇸

Palo Alto, California, United States

Children's Hospital of Michigan

🇺🇸

Detroit, Michigan, United States

Dana Faber Cancer Institute

🇺🇸

Boston, Massachusetts, United States

Seattle Cancer Care Alliance, University of Washington

🇺🇸

Seattle, Washington, United States

Hospital Das Clínicas da Faculdade de Medicina de Ribeirão Preto - USP

🇧🇷

Ribeirão Preto, Brazil

MHAT "Dr. Nikola Vasiliev" AD

🇧🇬

Kyustendil, Bulgaria

GSH Banco de Sangue de São Paulo

🇧🇷

São Paulo, Brazil

SHATHD Sofia

🇧🇬

Sofia, Bulgaria

Hôpital Necker Enfants Malades

🇫🇷

Paris, France

Hôpital Edouard Herriot, CHU de Lyon

🇫🇷

Lyon, France

Toronto General Hospital, University Health Network

🇨🇦

Toronto, Ontario, Canada

Rigshospitalet

🇩🇰

Hovedstaden, Denmark

CHU Hôpital de la Timone

🇫🇷

Marseille, France

CHU Hôpital Henri Mondor

🇫🇷

Créteil, France

Universitätsklinikum Essen

🇩🇪

Essen, Germany

Charité - UB - CVK - Medizinische Klinik

🇩🇪

Berlin, Germany

Universitätsklinikum Leipzig

🇩🇪

Leipzig, Germany

University General Hospital of Patras

🇬🇷

Achaia, Greece

Laiko General Hospital

🇬🇷

Athina, Greece

Ospedale "A. Perrino" - Brindisi

🇮🇹

Brindisi, Italy

Ospedale Sant'Anna

🇮🇹

Ferrara, Italy

Ente Ospedaliero Ospedali Galliera

🇮🇹

Genova, Italy

Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico

🇮🇹

Milano, Italy

Chronic Care Center

🇱🇧

Beyrouth, Lebanon

Hospital Sultanah Aminah Johor Bahru

🇲🇾

Johor Bahru, Malaysia

Hospital Sultanah Bahiyah

🇲🇾

Kota Setar, Malaysia

Hospital Umum Sarawak

🇲🇾

Kuching, Malaysia

Hospital Tunku Azizah

🇲🇾

Kuala Lumpur, Malaysia

King Abdulaziz Hospital - Al Ahsa

🇸🇦

Al-Ahsa, Saudi Arabia

Hospital Universitario Vall d'Hebron

🇪🇸

Barcelona, Spain

Hospital Universitario La Paz

🇪🇸

Madrid, Spain

Hospital Universitario Virgen Arrixaca

🇪🇸

Murcia, Spain

Hospital Universitario Virgen del Rocío

🇪🇸

Sevilla, Spain

National Taiwan University Hospital

🇨🇳

Taipei, Taiwan

Faculty of Medicine Siriraj Hospital

🇹🇭

Bangkok, Thailand

Naresuan University Hospital

🇹🇭

Mueang Phitsanulok, Thailand

Srinagarind Hospital, Khon Kaen University

🇹🇭

Mueang Khon Kaen, Thailand

Akdeniz University Faculty of Medicine

🇹🇷

Antalya, Turkey

Çukurova University

🇹🇷

Balcali, Turkey

Ege University Faculty of Medicine

🇹🇷

Bornova, Turkey

Hacettepe University

🇹🇷

Mersin, Turkey

Istanbul University Faculty of Medicine

🇹🇷

Fatih, Turkey

University College London

🇬🇧

London, United Kingdom

Weill Cornell Medical Center

🇺🇸

New York, New York, United States

San Diego Hospital, UC San Diego Health

🇺🇸

La Jolla, California, United States

Duke University Medical Center

🇺🇸

Durham, North Carolina, United States

Children's Hospital Agia Sophia, National and Kapodistrian University of Athens Medical School

🇬🇷

Athens, Greece

Hospital Queen Elizabeth, Kota Kinabalu

🇲🇾

Kota Kinabalu, Malaysia

A.O.U Di Modena

🇮🇹

Modena, Italy

A.O.U. San Luigi Gonzaga

🇮🇹

Orbassano, Italy

AOU L. Vanvitelli Universita degli Studi della Campania Luigi Vanvitelli

🇮🇹

Napoli, Italy

Acibadem Adana Hospital

🇹🇷

Adana, Turkey

Imperial College Healthcare NHS Trust - Hammersmith Hospital

🇬🇧

London, United Kingdom

UMHAT "Sveti Georgi" EAD

🇧🇬

Plovdiv, Bulgaria

Universitair Medisch Centrum Utrecht

🇳🇱

Utrecht, Netherlands

UMHAT "Prof. Dr. Stoyan Kirkovich"

🇧🇬

Stara Zagora, Bulgaria

Hospital Tengku Ampuan Afzan

🇲🇾

Kuantan, Malaysia

Foothills Medical Centre

🇨🇦

Calgary, Alberta, Canada

UMHAT "Dr. Georgi Stranski" Pleven

🇧🇬

Pleven, Bulgaria

Erasmus MC

🇳🇱

Westzeedijk 353, Netherlands

Maharaj Nakorn Chiang Mai Hospital

🇹🇭

Chiang Mai, Thailand

Hospital Ampang

🇲🇾

Pandan Indah, Malaysia

Amsterdam Universitair Medisch Centrum, Locatie AMC

🇳🇱

Amsterdam, Netherlands

Burjeel Medical City

🇦🇪

Abu Dhabi, United Arab Emirates

University Hospital of Ioannina

🇬🇷

Ioannina, Greece

Ippokrateio General Hospital

🇬🇷

Thessaloníki, Greece

Hospital Pulau Pinang

🇲🇾

Pulau Pinang, Malaysia

King Abdullah International Medical Research Center

🇸🇦

Riyadh, Saudi Arabia

King Khalid University Hospital

🇸🇦

Riyadh, Saudi Arabia

Changhua Christian Hospital

🇨🇳

Changhua, Taiwan

Phramongkutklao Hospital

🇹🇭

Bangkok, Thailand

Ramathibodi Hospital

🇹🇭

Bangkok, Thailand

King Chulalongkorn Memorial Hospital

🇹🇭

Pathum Wan, Thailand

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