An Efficacy and Safety Study of Luspatercept (ACE-536) Versus Placebo in Adults Who Require Regular Red Blood Cell Transfusions Due to Beta (β) Thalassemia

NCT ID: NCT02604433

Last Updated: 2023-04-18

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

336 participants

Study Classification

INTERVENTIONAL

Study Start Date

2016-05-02

Study Completion Date

2021-01-05

Brief Summary

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This is a Phase 3, double-blind, randomized, placebo-controlled, multicenter study to determine the efficacy and safety of luspatercept (ACE-536) plus Best supportive care (BSC) versus placebo plus BSC in adults who require regular red blood cell transfusion due to (β)-thalassemia.

The study is divided into the following periods:

* Historical Period,
* Screening/Run-in Period,
* Double-blind Treatment Period (48 weeks),
* Double-blind Long-term Treatment Period, (at the investigator's discretion an additional 48 weeks),
* Open-Label Phase post unblinding and upon Data Monitoring Committee positive recommendation
* Post-treatment Follow-up Period

Detailed Description

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Conditions

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Erythrocyte Transfusion Beta-Thalassemia

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Luspatercept (ACE-536) plus Best Supportive Care (BSC)

Luspatercept, subcutaneous(ly) (SC) once every 21 days

Group Type EXPERIMENTAL

Luspatercept

Intervention Type DRUG

Subjects will start with luspatercept at 1 mg/kg dose level.

Placebo plus Best Supportive Care (BSC)

normal saline solution subcutaneous(ly) (SC) once every 21 days

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type OTHER

Placebo, Subcutaneous, every 21 days.

Interventions

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Luspatercept

Subjects will start with luspatercept at 1 mg/kg dose level.

Intervention Type DRUG

Placebo

Placebo, Subcutaneous, every 21 days.

Intervention Type OTHER

Other Intervention Names

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ACE-536

Eligibility Criteria

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Inclusion Criteria

Subjects must satisfy the following criteria to be enrolled in the study:

1. Male or female, ≥18 years of age at the time of signing the informed consent document (ICF).
2. Subject must understand and voluntarily sign an Inform Consent Form prior to any study-related assessments/procedures being conducted.
3. Subject is willing and able to adhere to the study visit schedule and other protocol requirements.
4. Documented diagnosis of β-thalassemia or Hemoglobin E/β-thalassemia. (β-thalassemia with mutation and/or multiplication of alpha globin is allowed).
5. Regularly transfused, defined as: 6-20 Red Blood Cell (RBC) units\* in the 24 weeks prior to randomization and no transfusion-free period for ≥ 35 days during that period.

\* Sites who prescribe transfusions and have the transfusion records only in volumes should use for conversion of volume to units the below criteria, in order to obtain number of units within the last 24 weeks to assess the eligibility: 1 unit in this protocol refers to a quantity of packed RBCs approximately 200-350 mL. (i) sites who use transfusion bags within this range, or ≥ 350 mL, the conversion in units should be done by dividing the volume transfused to the patient by 350 mL, (ii) sites who use transfusion bags \< 200 mL, the conversion in units should be done by dividing the volume transfused to the patient by 200 mL.
6. Performance status: Eastern Cooperative Oncology Group (ECOG) score of 0 or 1.
7. A female of childbearing potential (FCBP) for this study is defined as a female who: 1) has achieved menarche at some point, 2) has not undergone a hysterectomy or bilateral oophorectomy or 3) has not been naturally postmenopausal (amenorrhea following cancer therapy does not rule out childbearing potential) for at least 24 consecutive months (ie, has had menses at any time in the preceding 24 consecutive months). FCBP participating in the study must:

1. Have two negative pregnancy tests as verified by the Investigator prior to starting study therapy. She must agree to ongoing pregnancy testing during the course of the study, and after end of study treatment. This applies even if the subject practices true abstinence \*\* from heterosexual contact.
2. Either commit to true abstinence\*\* from heterosexual contact (which must be reviewed on a monthly basis and source documented) If a FCBP engages in sexual activity that may result in a pregnancy, she must agree to use, and be able to comply with, effective\*\*\* contraception without interruption, 28 days prior to starting investigational product, during the study therapy (including dose interruptions), and for 12 weeks (approximately five times the mean terminal half-life of luspatercept based on multiple-dose Pharmacokinetic PK) data) after discontinuation of study therapy.

* True abstinence is acceptable when this is in line with the preferred and usual lifestyle of the subject. \[Periodic abstinence (eg, calendar, ovulation, symptothermal, post-ovulation methods) and withdrawal are not acceptable methods of contraception.\] \*\*\* Agreement to use highly effective methods of contraception that alone or in combination result in a failure rate of a Pearl index of less than 1% per year when used consistently and correctly throughout the course of the study.

Such methods include: Combined (estrogen and progesterone/progestin containing) hormonal contraception: Oral; Intravaginal; Transdermal; Progestogen/progestin only hormonal contraception associated with inhibition of ovulation: Oral; Injectable hormonal contraception; Implantable hormonal contraception; Placement of an intrauterine device (IUD); Placement of an intrauterine hormone-releasing system (IUS); Bilateral tubal occlusion; Vasectomized partner; Sexual Abstinence.
8. Male subjects must:

* Practice true abstinence or agree to use a condom during sexual contact with a pregnant female or a female of childbearing potential while participating in the study, during dose interruptions and for at least 12 weeks (approximately five times the mean terminal half-life of luspatercept based on multiple-dose PK data) following investigational product discontinuation, even if he has undergone a successful vasectomy.

Exclusion Criteria

The presence of any of the following will exclude a subject from enrollment:

1. Any significant medical condition, laboratory abnormality, or psychiatric illness that would prevent the subject from participating in the study.
2. Any condition including the presence of laboratory abnormalities, which places the subject at unacceptable risk if he/she were to participate in the study.
3. Any condition that confounds the ability to interpret data from the study.
4. A diagnosis of Hemoglobin S/β-thalassemia or alpha (α)-thalassemia (eg, Hemoglobin H);
5. Evidence of active hepatitis C (HCV) infection as demonstrated by a positive HCV-RNA test of sufficient sensitivity, or active infectious hepatitis B as demonstrated by the presence of HBsAg and/or HBVDNA-positive,, or known positive human immunodeficiency virus (HIV).

Note: Subjects receiving antiviral therapies should have 2 negative HCVRNA tests 3 months apart.(ie, one test at the end of the antiviral therapy and a second test 3 months following the first test).
6. Deep Vein Thrombosis (DVT) or stroke requiring medical intervention ≤ 24 weeks prior to randomization.
7. Use of chronic anticoagulant therapy is excluded, unless the treatment stopped at least 28 days prior to randomization. Anticoagulant therapies used for prophylaxis for surgery or high risk procedures as well as low Molecular Weight (LMW) heparin for superficial venous thrombosis and chronic aspirin are allowed.
8. Platelet count \> 1000 x 109/L
9. Poorly controlled diabetes mellitus within 24 weeks prior to randomization as defined by short term (eg, hyperosmolar or ketoacidotic crisis) and/or history of diabetic cardiovascular complications (eg, stroke or myocardial infarction).
10. Treatment with another investigational drug or device ≤ 28 days prior to randomization.
11. Prior exposure to sotatercept (ACE-011) or luspatercept (ACE-536).
12. Use of an erythropoiesis-stimulating agent (ESA) ≤ 24 weeks prior to randomization.
13. Iron chelation therapy, if initiated ≤ 24 weeks prior to randomization (allowed if initiated \> 24 weeks before or during treatment).
14. Hydroxyurea treatment ≤ 24 weeks prior to randomization.
15. Pregnant or lactating females.
16. Uncontrolled hypertension. Controlled hypertension for this protocol is considered ≤ Grade 1 according to NCI Common Terminology Criteria for Adverse Events (CTCAE) version 4.0 (current active minor version).
17. Major organ damage, including:

1. Liver disease with alanine aminotransferase (ALT) \> 3 x the upper limit of normal (ULN) or history of evidence of cirrhosis;
2. Heart disease, heart failure as classified by the New York Heart Association (NYHA) classification 3 or higher, or significant arrhythmia requiring treatment, or recent myocardial infarction within 6 months of randomization.
3. Lung disease, including pulmonary fibrosis or pulmonary hypertension which are clinically significant ie, ≥ Grade 3 NCI CTCAE version 4.0 (current active minor version).
4. Creatinine clearance \< 60 mL/min (per Cockroft-Gault formula).
18. Proteinuria ≥ Grade 3 according to NCI CTCAE version 4.0 (current active minor version).
19. Chronic systemic glucocorticoids ≤ 12 weeks prior to randomization (physiologic replacement therapy for adrenal insufficiency is allowed). Single day glucocorticoid treatment (eg, for prevention or treatment of transfusion reactions, is allowed).
20. Major surgery ≤ 12 weeks prior to randomization (subjects must have completely recovered from any previous surgery prior to randomization).
21. History of severe allergic or anaphylactic reactions or hypersensitivity to recombinant proteins or excipients in the investigational product (see Investigator Brochure).
22. Cytotoxic agents, immunosuppressants ≤ 28 days prior to randomization (ie, antithymocite globulin (ATG) or cyclosporine)
23. History of malignancy with the exception of:

1. Curatively resected nonmelanoma skin cancer.
2. Curatively treated cervical carcinoma in situ.
3. Other solid tumor with no known active disease in the opinion of the investigator.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Acceleron Pharma, Inc., a wholly-owned subsidiary of Merck & Co., Inc., Rahway, NJ USA

INDUSTRY

Sponsor Role collaborator

Celgene

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Bristol-Myers Squibb

Role: STUDY_DIRECTOR

Bristol-Myers Squibb

Locations

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Children's Hospital of Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital and Research Center at Oakland

Oakland, California, United States

Site Status

Ann and Robert H Lurie Childrens Hospital of Chicago

Chicago, Illinois, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

Weill Cornell Medical College

New York, New York, United States

Site Status

Hospital of the University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status

Prince of Wales Hospital

Randwick, New South Wales, Australia

Site Status

Mater Hospital Brisbane

South Brisbane, Queensland, Australia

Site Status

Royal Adelaide Hospital Institute of Medical and Veterinary Science

Adelaide, South Australia, Australia

Site Status

Monash Medical Centre

Clayton, Victoria, Australia

Site Status

Sir Charles Gairdner Hospital

Nedlands, Western Australia, Australia

Site Status

Royal Prince Alfred Hospital

Camperdown, , Australia

Site Status

University Mulitiprofile Hospital for Active Treatment Sveti Georgi EAD

Plovdiv, , Bulgaria

Site Status

Specialized Hospital for Active Treatment of Haematological Diseases - Sofia

Sofia, , Bulgaria

Site Status

Multiprofile Hospital for Active Treatment Sveta Marina EAD

Varna, , Bulgaria

Site Status

University Health Network

Toronto, Ontario, Canada

Site Status

Hopital Henri Mondor

Créteil, , France

Site Status

GH de Institut Catholique St. VincentHématologie

Lille, , France

Site Status

Hopitaux de La Timone

Marseille, , France

Site Status

Hospital of Necker

Paris, , France

Site Status

Laiko General Hospital of Athens

Ampelokipi - Athens, , Greece

Site Status

Local Institution - 405

Ampelokipi - Athens, , Greece

Site Status

General Children's Hospital "Agia Sophia"

Athens, , Greece

Site Status

General Hospital Georgios Gennimatas of Athens

Athens, , Greece

Site Status

University General Hospital of Patras

Rio Patras, , Greece

Site Status

Hippokration Hospital

Thessaloniki, , Greece

Site Status

Local Institution - 404

Thessaloniki, , Greece

Site Status

Soroka University Medical Centre

Beersheba, , Israel

Site Status

Rambam Health Corporation

Haifa, , Israel

Site Status

HaEmek Medical Center

Haïfa (Afula), , Israel

Site Status

Shaare Zedek Medical Center

Jerusalem, , Israel

Site Status

Hadassah Medical Center

Jerusalem, , Israel

Site Status

Galilee Medical Center

Nahariya, , Israel

Site Status

Rabin Medical Center

Petah Tikva, , Israel

Site Status

Presidio Ospedaliero Antonio Perrino

Brindisi, , Italy

Site Status

Universita degli Studi di Cagliari - ASL8

Cagliari, , Italy

Site Status

Azienda Ospedaliero-Universitaria di Ferrara - Arcispedale Sant'Anna

Ferrara, , Italy

Site Status

Ente Ospedaliero Ospedali Galliera - Centro della Microcitemia e delle Anemie Congenite

Genoa, , Italy

Site Status

Fondazione Ca Granda IRCCS Ospedale Maggiore

Milan, , Italy

Site Status

Seconda Universita Degli Studi Di Napoli

Naples, , Italy

Site Status

AORN A Cardarelli

Napoli, , Italy

Site Status

Azienda Ospedaliero Universitaria S. Luigi Gonzaga

Orbassano, , Italy

Site Status

Azienda Ospedaliera Ospedali Riuniti Villa Sofia-Cervello

Palermo, , Italy

Site Status

Azienda Ospedaliera Universitaria Integrata Di Verona

Verona, , Italy

Site Status

Chronic Care Center

Beirut, , Lebanon

Site Status

Hospital Sultanah Aminah

Johor Bahru, Johor, Malaysia

Site Status

Hospital Sultanah Bahiyah

Alor Star, Kedah, Malaysia

Site Status

University Malaya Medical Centre

Kuala Lumpur, Kuala Lumpur, Malaysia

Site Status

Hospital Raja Permaisuri Bainun

Ipoh, Perak, Malaysia

Site Status

Queen Elizabeth Hospital

Kota Kinabalu, Sabah, Malaysia

Site Status

Hospital Umum Sarawak

Kuching, Sarawak, Malaysia

Site Status

Hospital Pulau Pinang c/o Penang Medical College

George Town, , Malaysia

Site Status

Changhua Christian Hospital

Changhua, , Taiwan

Site Status

Kaohsiung Medical University Hospital

Kaohsiung City, , Taiwan

Site Status

China Medical University Hospital

Taichung, , Taiwan

Site Status

National Taiwan University Hospital

Taipei, Zhongzheng Dist., , Taiwan

Site Status

Chulalongkorn University Faculty of Medicine - King Chulalongkorn Memorial Hospital

Bangkok, , Thailand

Site Status

Siriraj Hospital Mahidol University

Bangkok, , Thailand

Site Status

Chiang Mai University - Maharaj Nakorn Chiang Mai Hospital

Chiang Mai, , Thailand

Site Status

University Hospital Farhat Hached

Sousse, , Tunisia

Site Status

Bone Marrow Transplant Center

Tunis, , Tunisia

Site Status

Aziza Othmana Hospital

Tunis, , Tunisia

Site Status

Military Hospital of Tunis

Tunis, , Tunisia

Site Status

Acibadem Adana Hospital

Adana, , Turkey (Türkiye)

Site Status

Cukurova University Medical Faculty Balcali Hospital

Adana, , Turkey (Türkiye)

Site Status

Local Institution - 524

Adana, , Turkey (Türkiye)

Site Status

Hacettepe Universitesi

Ankara, , Turkey (Türkiye)

Site Status

Antalya Egitim Arastirma

Antalya, , Turkey (Türkiye)

Site Status

Istanbul Universitesi Istanbul Tip Fakultesi Hastanesi

Istanbul, , Turkey (Türkiye)

Site Status

Ege Universitesi Tip Fakultesi Hastanesi

Izmir, , Turkey (Türkiye)

Site Status

Mersin University Medical Faculty

Mersin, , Turkey (Türkiye)

Site Status

St James University Hospital

Leeds, , United Kingdom

Site Status

Barts Health NHS Trust - The Royal London Hospital

London, , United Kingdom

Site Status

Whittington Hospital

London, , United Kingdom

Site Status

University College Hospital Trust

London Bloomsbury, , United Kingdom

Site Status

Manchester Royal Infirmary

Manchester, , United Kingdom

Site Status

Countries

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United States Australia Bulgaria Canada France Greece Israel Italy Lebanon Malaysia Taiwan Thailand Tunisia Turkey (Türkiye) United Kingdom

References

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Porter J. Beyond transfusion therapy: new therapies in thalassemia including drugs, alternate donor transplant, and gene therapy. Hematology Am Soc Hematol Educ Program. 2018 Nov 30;2018(1):361-370. doi: 10.1182/asheducation-2018.1.361.

Reference Type RESULT
PMID: 30504333 (View on PubMed)

Piga A, Perrotta S, Gamberini MR, Voskaridou E, Melpignano A, Filosa A, Caruso V, Pietrangelo A, Longo F, Tartaglione I, Borgna-Pignatti C, Zhang X, Laadem A, Sherman ML, Attie KM. Luspatercept improves hemoglobin levels and blood transfusion requirements in a study of patients with beta-thalassemia. Blood. 2019 Mar 21;133(12):1279-1289. doi: 10.1182/blood-2018-10-879247. Epub 2019 Jan 7.

Reference Type RESULT
PMID: 30617198 (View on PubMed)

Cappellini MD, Viprakasit V, Georgiev P, Coates TD, Origa R, Khelif A, Liew HK, Tantiworawit A, Chew LP, Khalil A, Ho PJ, Kuo KHM, Holot N, Perin M, Giuseppi AC, Kuo WL, Lai Y, Medlin LF, Bueno LM, Kattamis A, Taher AT. Long-term efficacy and safety of luspatercept for the treatment of anaemia in patients with transfusion-dependent beta-thalassaemia (BELIEVE): final results from a phase 3 randomised trial. Lancet Haematol. 2025 Mar;12(3):e180-e189. doi: 10.1016/S2352-3026(24)00376-4. Epub 2025 Feb 10.

Reference Type DERIVED
PMID: 39947215 (View on PubMed)

Garbowski MW, Ugidos M, Risueno A, Shetty JK, Schwickart M, Hermine O, Porter JB, Thakurta A, Vodala S. Luspatercept stimulates erythropoiesis, increases iron utilization, and redistributes body iron in transfusion-dependent thalassemia. Am J Hematol. 2024 Feb;99(2):182-192. doi: 10.1002/ajh.27102. Epub 2023 Oct 2.

Reference Type DERIVED
PMID: 37782758 (View on PubMed)

Denton CC, Vodala S, Veluswamy S, Hofstra TC, Coates TD, Wood JC. Splenic iron decreases without change in volume or liver parameters during luspatercept therapy. Blood. 2023 Nov 30;142(22):1932-1934. doi: 10.1182/blood.2023021839.

Reference Type DERIVED
PMID: 37704579 (View on PubMed)

Cappellini MD, Taher AT. The use of luspatercept for thalassemia in adults. Blood Adv. 2021 Jan 12;5(1):326-333. doi: 10.1182/bloodadvances.2020002725.

Reference Type DERIVED
PMID: 33570654 (View on PubMed)

Cappellini MD, Viprakasit V, Taher AT, Georgiev P, Kuo KHM, Coates T, Voskaridou E, Liew HK, Pazgal-Kobrowski I, Forni GL, Perrotta S, Khelif A, Lal A, Kattamis A, Vlachaki E, Origa R, Aydinok Y, Bejaoui M, Ho PJ, Chew LP, Bee PC, Lim SM, Lu MY, Tantiworawit A, Ganeva P, Gercheva L, Shah F, Neufeld EJ, Thompson A, Laadem A, Shetty JK, Zou J, Zhang J, Miteva D, Zinger T, Linde PG, Sherman ML, Hermine O, Porter J, Piga A; BELIEVE Investigators. A Phase 3 Trial of Luspatercept in Patients with Transfusion-Dependent beta-Thalassemia. N Engl J Med. 2020 Mar 26;382(13):1219-1231. doi: 10.1056/NEJMoa1910182.

Reference Type DERIVED
PMID: 32212518 (View on PubMed)

Provided Documents

Download supplemental materials such as informed consent forms, study protocols, or participant manuals.

Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Related Links

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Other Identifiers

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ACE-536-B-THAL-001

Identifier Type: -

Identifier Source: org_study_id

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