NCT02766465

Brief Summary

This is a clinical trial that will compare survival and sickle related outcomes in adolescents and young adults with severe sickle cell disease after bone marrow transplantation and standard of care. The primary outcome is 2-year overall survival.

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
138

participants targeted

Target at P75+ for phase_2

Timeline
Completed

Started Mar 2017

Longer than P75 for phase_2

Geographic Reach
1 country

35 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

May 6, 2016

Completed
3 days until next milestone

First Posted

Study publicly available on registry

May 9, 2016

Completed
10 months until next milestone

Study Start

First participant enrolled

March 16, 2017

Completed
6.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 2, 2023

Completed
3 months until next milestone

Study Completion

Last participant's last visit for all outcomes

August 2, 2023

Completed
1.1 years until next milestone

Results Posted

Study results publicly available

September 24, 2024

Completed
Last Updated

April 18, 2025

Status Verified

April 1, 2025

Enrollment Period

6.1 years

First QC Date

May 6, 2016

Results QC Date

June 7, 2024

Last Update Submit

April 11, 2025

Conditions

Keywords

Sickle Cell DiseaseYoung AdultsPhase II TrialHematopoietic Cell Transplantation (HCT)Human Leukocyte Antigen (HLA)

Outcome Measures

Primary Outcomes (1)

  • Percentage of Participants With Overall Survival (OS) at 2 Years After Biologic Assignment

    Due to incomplete accrual, there was not adequate statistical power to analyze the primary endpoint as specified. Instead, point estimates for the observed proportion of patients surviving at two years post-biologic assignment in each arm were generated descriptively, with 95% CI, using the Kaplan Meier methodology. The event of interest was death from any cause.

    2 Years

Secondary Outcomes (29)

  • Frequencies of Sickle Cell Disease (SCD) Events of Special Interest

    2 Years

  • Change in 6-minute Walk Distance (6MWD) Assessment From Baseline

    Baseline, 2 years

  • Change in Transcuspid Regurgitant Jet Velocity (TRJV) Assessment From Baseline

    Baseline, 2 years

  • Change in Albuminuria Assessment From Baseline

    Baseline, 2 years

  • Health-Related Quality of Life (HRQoL) Component Physical Function Changes From Baseline

    Baseline, 2 years

  • +24 more secondary outcomes

Study Arms (2)

Donor Arm

EXPERIMENTAL

Donor Arm patients will undergo hematopoietic cell transplant. Patients with a matched unrelated donor will receive a bone marrow transplant (unless PBSC graft is pre-approved per section 2.5.1 using a preparative regimen with Busulfan, Fludarabine and rabbit ATG. Patients with an HLA-identical sibling donor can receive a transplant using one of three regimens: A. Busulfan, Fludarabine, and rabbit ATG using a bone marrow graft (preferred regimen) B. Alemtuzumab/TBI 300 cGy using a peripheral blood graft C. Alemtuzumab, fludarabine, melphalan using a bone marrow graft

Drug: BusulfanDrug: FludarabineDrug: r-ATGProcedure: Hematopoietic Cell TransplantDrug: TacrolimusDrug: MethotrexateDrug: AlemtuzumabDrug: Total Body Irradiation (TBI)Drug: SirolimusDrug: MelphalanDrug: G-CSF

No-Donor Arm

ACTIVE COMPARATOR

No-donor arm patients will continue with standard of care per their SCD physician.

Procedure: Standard of Care

Interventions

A: Busulfan dose will be 3.2 mg/kg administered as a single daily dose IV on days -8 through -5 with dosing adjusted using targeted pharmacokinetics.

Also known as: Busulfex
Donor Arm

A: Fludarabine dose will be 35 mg/m\^2/day administered IV on days -7 through -3 (total fludarabine dose is 175 mg/m\^2). C: Fludarabine 30mg/m2 IV dose will be given on Days -8, -7, -6, -5, -4

Also known as: Fludara
Donor Arm
r-ATGDRUG

A: r-ATG will be administered IV on day -6 at 0.5mg/kg, on day -5 at 1 mg/kg and on days -4, -3 and -2 at 1.5mg/kg (total r-ATG dose is 6 mg/kg).

Also known as: Rabbit antithymocyte globulin
Donor Arm

A,B,C: Day 0 is the day of transplantation.

Also known as: Bone Marrow Transplant; BMT; HCT
Donor Arm

A: Tacrolimus commences on day -3 and extends through day +180 after transplantation with doses adjusted to maintain appropriate levels according to institutional guidelines. C: Tacrolimus at therapeutic doses through Day 180, then taper per institutional guidelines

Also known as: Prograf®
Donor Arm

A: Methotrexate will be administered intravenously on day+1 at 15mg/m\^2, day+3 at 10mg/m\^2, day+6 at 10mg/m\^2, and day+11 at 10mg/m\^2. C: Methotrexate IV 7.5 mg/m2 dose will be given on Days +1, 3, +6 following transplant

Also known as: MTX
Donor Arm

Continue to receive standard of care treatment per patient's SCD physician.

No-Donor Arm

B: Alemtuzumab 0.03 mg/kg IV dose will be given on Day -7, Alemtuzumab 0.1 mg/kg IV dose will be given on Day -6, Alemtuzumab 0.3 mg/kg IV dose will be given on Day -5,-4,-3 C: Alemtuzumab test dose 3 mg IV once 24 hours prior to 1st dose of Alemtuzumab Alemtuzumab 10 mg IV, 15 mg IV, 20 mg IV given on Days -22 through Day -18. Alemtuzumab doses may be administered between Days -22 and -18 but are required to be on three consecutive days.

Also known as: Lemtrada
Donor Arm

Total Body Irradiation 300 cGY on Day -2

Donor Arm

Sirolimus at therapeutic doses through day 180, then taper per institutional guidelines if donor CD3+ \>50%

Also known as: Rapamune
Donor Arm

C: Melphalan 140 mg/m2 IV dose will be given on Day -3

Also known as: Alkeran
Donor Arm
G-CSFDRUG

G-CSF 5 μg/kg/day continue until neutrophil engraftment.

Also known as: Granulocyte colony-stimulating factor
Donor Arm

Eligibility Criteria

Age15 Years - 40 Years
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64)

You may qualify if:

  • Age ≥ 15 and \< 41 years
  • Severe sickle cell disease \[Hemoglobin SS (Hb SS), Hemoglobin SC (Hb SC) or Hemoglobin SBeta thalassemia (Hb Sβ) genotype\] with at least 1 of the following manifestations (a-e):
  • Clinically significant neurologic event (stroke) or any neurological deficit lasting \> 24 hours;
  • History of two or more episodes of acute chest syndrome (ACS) in the 2-year period preceding enrollment despite the institution of supportive care measures (i.e. asthma therapy);
  • An average of three or more pain crises per year in the 2-year period preceding enrollment (required intravenous pain management in the outpatient or inpatient hospital setting). Clinical documentation of pain management in the inpatient or outpatient setting is required.
  • Administration of regular RBC transfusion therapy, defined as receiving 8 or more transfusion events per year (in the 12 months before enrollment) to prevent vaso-occlusive clinical complications (i.e. pain, stroke, and acute chest syndrome)
  • An echocardiographic finding of tricuspid valve regurgitant jet (TRJ) velocity ≥ 2.7 m/sec.
  • Ongoing high impact chronic pain on a majority of days per month for ≥ 6 months as defined as ONE or more of the following: Chronic pain without contributory SCD complications, OR Mixed pain type in which chronic pain is occurring at site(s) (arms, back, chest, or abdominal pain) unrelated to any sites associated with Contributory SCD complications (e.g. leg ulcers and/or avascular necrosis).
  • i. High impact chronic pain is identified as those reporting "severe interference" with life activities OR "usually or always" experiencing a limitation of their life or work activities including household chores. (See guidelines for identifying HICP in the BMT CTN 1503 Manual of Procedures) ii. Contributory SCD complications are defined as clinical signs (e.g. presence of leg ulcers) or clinical assessments (e.g. imaging confirmation of splenic infarct or avascular necrosis). Chronic pain attributed solely to contributory SCD complications is excluded.
  • Adequate physical function as measured by all of the following:
  • Karnofsky/Lansky performance score ≥ 60
  • Cardiac function: Left ventricular ejection fraction (LVEF) \> 40%; or LV shortening fraction \> 26% by cardiac echocardiogram or by Multi Gated Acquisition Scan (MUGA).
  • Pulmonary function:
  • a. Pulse oximetry with a baseline O2 saturation of ≥ 85% b. Diffusing capacity of the lung for carbon monoxide (DLCO) \> 40% (corrected for hemoglobin) d. Renal function: Serum creatinine ≤ 1.5 x the upper limit of normal for age as per local laboratory and 24 hour urine creatinine clearance \>70 mL/min; or GFR \> 70 mL/min/1.73 m2 by radionuclide Glomerular Filtration Rate (GFR).
  • e. Hepatic function:
  • +5 more criteria

You may not qualify if:

  • HLA typing with a donor search prior to referral (consultation with HCT physician).
  • If a subject has had HLA typing and a related donor search that did not identify a suitably matched relative (i.e., sibling) at any time, and also did not have an unrelated donor search, the patient will be considered eligible.
  • If a subject has had HLA typing and a related donor search that did not identify a suitably matched relative (i.e., sibling) at any time and had an unrelated donor search that did not identify a suitably matched unrelated donor ≥ 1 year prior to enrollment, the patient will be considered eligible.
  • If a subject has had HLA typing with no related donor search and had an unrelated donor search that did not identify a suitably matched unrelated donor ≥ 1 year prior to enrollment, the patient will be considered eligible.
  • Subjects with a known HLA-identical sibling or HLA-matched unrelated donor are excluded
  • Uncontrolled bacterial, viral or fungal infection in the 6 weeks before enrollment.
  • Seropositivity for HIV.
  • Previous HCT or solid organ transplant.
  • Participation in a clinical trial in which the patient received an investigational drug or device must be discontinued at enrollment.
  • A history of substance abuse as defined by version IV of the Diagnostic \& Statistical Manual of Mental Disorders (DSM IV).
  • Demonstrated lack of compliance with prior medical care as determined by referring physician.
  • Pregnant or breast feeding females.
  • Inability to receive HCT due to alloimmunization, defined as the inability to receive packed red blood cell (pRBC) transfusion therapy.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (35)

Benioff Children's Hospital at Oakland

Oakland, California, 94609, United States

Location

Children's National Medical Center

Washington D.C., District of Columbia, 20010, United States

Location

University of Florida Gainsville

Gainesville, Florida, 32611, United States

Location

Foundation for Sickle Cell Research/Florida Sickle Inc.

Hollywood, Florida, 33021, United States

Location

University of Miami

Miami, Florida, 33136, United States

Location

Grady Memorial Hospital

Atlanta, Georgia, 30303, United States

Location

Children's Healthcare of Atlanta

Atlanta, Georgia, 30322, United States

Location

Emory University

Atlanta, Georgia, 30322, United States

Location

Augusta University Medical Center

Augusta, Georgia, 30912, United States

Location

University of Iowa

Iowa City, Iowa, 52242, United States

Location

Children's Hospital of New Orleans

New Orleans, Louisiana, 70118, United States

Location

Dana Farber Cancer Institute/Brigham & Women's Hospital

Boston, Massachusetts, 02114, United States

Location

Dana Farber Cancer Institute/Massachusetts General Hospital

Boston, Massachusetts, 02115, United States

Location

Boston University

Boston, Massachusetts, 02215, United States

Location

Barbara Ann Karmanos Cancer Institute

Detroit, Michigan, 48201, United States

Location

Washington University/St. Louis Children's Hospital

St Louis, Missouri, 63110, United States

Location

Hackensack University Medical Center

Hackensack, New Jersey, 07601, United States

Location

Newark Beth Israel Medical Center

Newark, New Jersey, 07112, United States

Location

New York Presbyterian Brooklyn Methodist Hospital

Brooklyn, New York, 11215, United States

Location

Cohen Children's Medical Center

New Hyde Park, New York, 11040, United States

Location

Icahn School of Medicine at Mount Sinai

New York, New York, 10029, United States

Location

Weill Cornell Medical College

New York, New York, 10065, United States

Location

Montefiore Medical Center/Albert Einstein School of Medicine

The Bronx, New York, 10467, United States

Location

University of North Carolina Hospital at Chapel Hill

Chapel Hill, North Carolina, 27516, United States

Location

Duke University Medical Center

Durham, North Carolina, 27705, United States

Location

Ohio State University

Columbus, Ohio, 43210, United States

Location

University of Oklahoma

Oklahoma City, Oklahoma, 73104, United States

Location

Oregon Health Sciences University

Portland, Oregon, 97239, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

Medical University of South Carolina

Charleston, South Carolina, 29425, United States

Location

University of Texas Health Sciences Center

Houston, Texas, 77004, United States

Location

Baylor College of Medicine/The Methodist Hospital

Houston, Texas, 77030, United States

Location

University of Texas/MD Anderson CRC

Houston, Texas, 77030, United States

Location

University of Virginia

Charlottesville, Virginia, 22908, United States

Location

Virginia Commonwealth University

Richmond, Virginia, 23298, United States

Location

Related Publications (3)

  • Walters MC, Eapen M, Liu Y, El Rassi F, Waller EK, Levine JE, Strouse JJ, Antin JH, Parikh SH, Bakshi N, Dampier C, Jaroscak JJ, Bergmann S, Wong T, Kota V, Pace B, Lekakis LJ, Lulla P, Nickel RS, Kasow KA, Popat U, Smith W, Yu L, DiFronzo N, Geller N, Kamani N, Klings ES, Hassell K, Mendizabal A, Sullivan K, Neuberg D, Krishnamurti L. Hematopoietic cell transplant compared with standard care in adolescents and young adults with sickle cell disease. Blood Adv. 2025 Mar 11;9(5):955-965. doi: 10.1182/bloodadvances.2024013926.

  • Leonard A, Furstenau D, Abraham A, Darbari DS, Nickel RS, Limerick E, Fitzhugh C, Hsieh M, Tisdale JF. Reduction in vaso-occlusive events following stem cell transplantation in patients with sickle cell disease. Blood Adv. 2023 Jan 24;7(2):227-234. doi: 10.1182/bloodadvances.2022008137.

  • Krishnamurti L, Neuberg DS, Sullivan KM, Kamani NR, Abraham A, Campigotto F, Zhang W, Dahdoul T, De Castro L, Parikh S, Bakshi N, Haight A, Hassell KL, Loving R, Rosenthal J, Smith SL, Smith W, Spearman M, Stevenson K, Wu CJ, Wiedl C, Waller EK, Walters MC. Bone marrow transplantation for adolescents and young adults with sickle cell disease: Results of a prospective multicenter pilot study. Am J Hematol. 2019 Apr;94(4):446-454. doi: 10.1002/ajh.25401. Epub 2019 Feb 11.

Related Links

MeSH Terms

Conditions

Anemia, Sickle Cell

Interventions

Busulfanfludarabinefludarabine phosphatethymoglobulinBone Marrow TransplantationHematocritTacrolimusMethotrexateStandard of CareAlemtuzumabWhole-Body IrradiationSirolimusMelphalanGranulocyte Colony-Stimulating Factor

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, CongenitalAnemia, HemolyticAnemiaHematologic DiseasesHemic and Lymphatic DiseasesHemoglobinopathiesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Intervention Hierarchy (Ancestors)

Butylene GlycolsGlycolsAlcoholsOrganic ChemicalsMesylatesAlkanesulfonatesAlkanesulfonic AcidsAlkanesHydrocarbons, AcyclicHydrocarbonsSulfonic AcidsSulfur AcidsSulfur CompoundsTissue TransplantationCell- and Tissue-Based TherapyBiological TherapyTherapeuticsTransplantationSurgical Procedures, OperativeHematologic TestsClinical Laboratory TechniquesDiagnostic Techniques and ProceduresDiagnosisInvestigative TechniquesHemorheologyBlood Physiological PhenomenaCirculatory and Respiratory Physiological PhenomenaMacrolidesLactonesAminopterinPterinsPteridinesHeterocyclic Compounds, 2-RingHeterocyclic Compounds, Fused-RingHeterocyclic CompoundsQuality Indicators, Health CareQuality of Health CareHealth Services AdministrationHealth Care Quality, Access, and EvaluationAntibodies, Monoclonal, HumanizedAntibodies, MonoclonalAntibodiesImmunoglobulinsImmunoproteinsBlood ProteinsProteinsAmino Acids, Peptides, and ProteinsSerum GlobulinsGlobulinsRadiotherapyNitrogen Mustard CompoundsMustard CompoundsHydrocarbons, HalogenatedPhenylalanineAmino Acids, AromaticAmino Acids, CyclicAmino AcidsColony-Stimulating FactorsGlycoproteinsGlycoconjugatesCarbohydratesHematopoietic Cell Growth FactorsCytokinesIntercellular Signaling Peptides and ProteinsPeptidesBiological Factors

Results Point of Contact

Title
Adam Mendizabal, PhD
Organization
The Emmes Company, LLC

Study Officials

  • Mary Eapen, MD

    Center for International Blood and Marrow Transplant Research

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restrictive Agreement
No

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
PARALLEL
Model Details: Patients are enrolled without a known donor. All patients have 180 days from confirmation of eligibility to confirm a donor. Patients with a donor will be assigned to the Donor Arm and receive HCT; patients without a donor will be assigned to the no donor arm and continue receiving standard of care for their SCD.
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

May 6, 2016

First Posted

May 9, 2016

Study Start

March 16, 2017

Primary Completion

May 2, 2023

Study Completion

August 2, 2023

Last Updated

April 18, 2025

Results First Posted

September 24, 2024

Record last verified: 2025-04

Data Sharing

IPD Sharing
Will share

Results will be published in a manuscript and supporting information submitted to NIH BioLINCC (including data dictionaries, case report forms, data submission documentation, documentation for outcomes dataset, etc where indicated).

Shared Documents
STUDY PROTOCOL, ICF
Time Frame
Within 6 months of official study closure at participating sites.
Access Criteria
Available to the public
More information

Locations