Alpha/Beta TCD HCT in Patients With Inherited BMF Disorders

NCT ID: NCT03579875

Last Updated: 2026-01-30

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

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

48 participants

Study Classification

INTERVENTIONAL

Study Start Date

2018-11-13

Study Completion Date

2029-01-05

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

This is a phase II trial of T cell receptor alpha/beta depletion (α/β TCD) peripheral blood stem cell (PBSC) transplantation in patients with inherited bone marrow failure (BMF) disorders to eliminate the need for routine graft-versus-host disease (GVHD) immune suppression leading to earlier immune recovery and potentially a reduction in the risk of severe infections after transplantation.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Fanconi Anemia Severe Aplastic Anemia Myelodysplastic Syndromes T Cell Receptor Alpha/Beta Depletion Telomere Biology Disorder Bone Marrow Failure Dyskeratosis Congenita Telomere Biology Disorders

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi Anemia

Given to:

* Patients with an unrelated donor or HLA mismatched related donor, regardless of disease type OR
* Patients with an HLA- identical sibling donor recipient and myelodysplastic features, MDS, or acute leukemia

Group Type EXPERIMENTAL

Total Body Irradiation (TBI) (Plan 1)

Intervention Type DRUG

300 cGy with thymic shielding on day -6

Cyclophosphamide (CY) (Plan 1)

Intervention Type DRUG

10 mg/kg IV daily on days -5, -4, -3, and -2

Fludarabine (FLU)

Intervention Type DRUG

35 mg/m2 IV daily on days -5, -4, -3, and -2

Methylprednisolone (MP)

Intervention Type DRUG

1 mg/kg IV q12h on days -5, -4, -3, -2, and -1

Donor mobilized PBSC infusion

Intervention Type DEVICE

T cell receptor alpha/beta depletion (α/β TCD) peripheral blood stem cell (PBSC) transplantation on day 0

G-CSF

Intervention Type DRUG

Initiate G-CSF 5mcg/kg per day IV on day +1 (continue until ANC \>2.5 x 10\^9/L for 3 consecutive days or single day ANC \>3000 Arm 1 and Arm 3)

Rituximab

Intervention Type DRUG

200 mg/m2 IV once on day -1

Rituximab

Intervention Type DRUG

Rituximab will be given once on treatment plans 1-3 on day -1.

Treatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi Anemia

Given to:

• An HLA-identical sibling donor recipients with single or multi- lineage hematopoietic failure

Group Type EXPERIMENTAL

Fludarabine (FLU)

Intervention Type DRUG

35 mg/m2 IV daily on days -5, -4, -3, and -2

Methylprednisolone (MP)

Intervention Type DRUG

1 mg/kg IV q12h on days -5, -4, -3, -2, and -1

Donor mobilized PBSC infusion

Intervention Type DEVICE

T cell receptor alpha/beta depletion (α/β TCD) peripheral blood stem cell (PBSC) transplantation on day 0

G-CSF

Intervention Type DRUG

Initiate G-CSF 5mcg/kg per day IV on day +1 (continue until ANC \>2.5 x 10\^9/L for 3 consecutive days or single day ANC \>3000 Arm 1 and Arm 3)

Cyclophosphamide (CY) (Plan 2)

Intervention Type DRUG

5 mg/kg IV daily on days -5, -4, -3, and -2

Rituximab

Intervention Type DRUG

200 mg/m2 IV once on day -1

Rituximab

Intervention Type DRUG

Rituximab will be given once on treatment plans 1-3 on day -1.

Treatment Plan 3: BU, Cy, FLU, MP and Rituximab in patients with Fanconi Anemia

Given to:

* Patients with an unrelated donor or HLA mismatched related donor, regardless of disease type who cannot tolerate TBI
* Patients with an HLA- identical sibling donor recipient and myelodysplastic features, MDS, or acute leukemia who cannot tolerate TBI
* Biallelic mutations in FANCD1/BRCA2 who cannot receive TBI
* Per treating physician preference

Group Type EXPERIMENTAL

Cyclophosphamide (CY) (Plan 1)

Intervention Type DRUG

10 mg/kg IV daily on days -5, -4, -3, and -2

Fludarabine (FLU)

Intervention Type DRUG

35 mg/m2 IV daily on days -5, -4, -3, and -2

Methylprednisolone (MP)

Intervention Type DRUG

1 mg/kg IV q12h on days -5, -4, -3, -2, and -1

Rituximab

Intervention Type DRUG

200 mg/m2 IV once on day -1

Busulfan

Intervention Type DRUG

Busulfan 0.6 mg/kg if \> 4 years old and/or \>12 kg (0.8 mg/kg IV if ≤ 4 years old and/or ≤ 12 kg) is given IV over 2 hours every 12 hours for 2 days.

Rituximab

Intervention Type DRUG

Rituximab will be given once on treatment plans 1-3 on day -1.

Treatment Plan 4: CY, FLU, and alemtuzumab

given to TBD patients with:

* Bone marrow failure AND
* Any donor type including haploidentical (4/8) to 8/8-HLA matched related donor, or 7-8/8 HLA-matched unrelated donor Based on historical numbers, it is expected approximately 3 patients would be treated per year. Statistical outcomes will be descriptive.

Group Type EXPERIMENTAL

Cyclophosphamide (CY) (Plan 1)

Intervention Type DRUG

10 mg/kg IV daily on days -5, -4, -3, and -2

Fludarabine (FLU)

Intervention Type DRUG

35 mg/m2 IV daily on days -5, -4, -3, and -2

Alemtuzumab

Intervention Type DRUG

Alemtuzumab 0.2 mg/kg is given IV over 2 hours daily for 5 days (total dose 1 mg/kg)

Treatment Plan 5: CY, FLU, melphalan (MEL), and alemtuzumab.

given to TBD patients with:

* Early myelodysplastic features (with or without cytogenetic abnormalities) AND
* Any donor type including haploidentical (4/8) to 8/8-HLA matched related donor, or 7-8/8 HLA-matched unrelated donor Based on historical numbers, it is expected approximately 2 patients would be treated per year. Statistical outcomes will be descriptive.

Group Type EXPERIMENTAL

Cyclophosphamide (CY) (Plan 1)

Intervention Type DRUG

10 mg/kg IV daily on days -5, -4, -3, and -2

Fludarabine (FLU)

Intervention Type DRUG

35 mg/m2 IV daily on days -5, -4, -3, and -2

Alemtuzumab

Intervention Type DRUG

Alemtuzumab 0.2 mg/kg is given IV over 2 hours daily for 5 days (total dose 1 mg/kg)

Melphalan

Intervention Type DRUG

If available, MEL dosing will be model-based using Bayesian methodology. If Bayesian methodology is unavailable, MEL dosing will be weight-based: MEL 70 mg/m2 for patients ≥10 kg (2.35 mg/kg for patients \<10 kg\^) IV for one dose over 30 minutes.

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Total Body Irradiation (TBI) (Plan 1)

300 cGy with thymic shielding on day -6

Intervention Type DRUG

Cyclophosphamide (CY) (Plan 1)

10 mg/kg IV daily on days -5, -4, -3, and -2

Intervention Type DRUG

Fludarabine (FLU)

35 mg/m2 IV daily on days -5, -4, -3, and -2

Intervention Type DRUG

Methylprednisolone (MP)

1 mg/kg IV q12h on days -5, -4, -3, -2, and -1

Intervention Type DRUG

Donor mobilized PBSC infusion

T cell receptor alpha/beta depletion (α/β TCD) peripheral blood stem cell (PBSC) transplantation on day 0

Intervention Type DEVICE

G-CSF

Initiate G-CSF 5mcg/kg per day IV on day +1 (continue until ANC \>2.5 x 10\^9/L for 3 consecutive days or single day ANC \>3000 Arm 1 and Arm 3)

Intervention Type DRUG

Cyclophosphamide (CY) (Plan 2)

5 mg/kg IV daily on days -5, -4, -3, and -2

Intervention Type DRUG

Rituximab

200 mg/m2 IV once on day -1

Intervention Type DRUG

Busulfan

Busulfan 0.6 mg/kg if \> 4 years old and/or \>12 kg (0.8 mg/kg IV if ≤ 4 years old and/or ≤ 12 kg) is given IV over 2 hours every 12 hours for 2 days.

Intervention Type DRUG

Alemtuzumab

Alemtuzumab 0.2 mg/kg is given IV over 2 hours daily for 5 days (total dose 1 mg/kg)

Intervention Type DRUG

Melphalan

If available, MEL dosing will be model-based using Bayesian methodology. If Bayesian methodology is unavailable, MEL dosing will be weight-based: MEL 70 mg/m2 for patients ≥10 kg (2.35 mg/kg for patients \<10 kg\^) IV for one dose over 30 minutes.

Intervention Type DRUG

Rituximab

Rituximab will be given once on treatment plans 1-3 on day -1.

Intervention Type DRUG

Other Intervention Names

Discover alternative or legacy names that may be used to describe the listed interventions across different sources.

TBI CY FLU MP PBSC CY BU MEL

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

For FA patients:

* Diagnosis of Fanconi anemia

* Age \<65 years of age
* Has one of the following risk factors:

* Severe aplastic anemia (SAA)
* Myelodysplastic features
* High risk genotype
* Immunodeficiency associated with history of recurrent infections
* Karnofsky performance status ≥ 70% if ≥ 16 years of age or Lansky play score ≥ 50% for patients \<16 years of age

* Adequate pulmonary, cardiac and liver function
* Voluntary written consent (minor assent if appropriate) prior to the performance of any study related procedures not part of standard medical care

For TBD patients:

• Diagnosis of TBD

* Age \<70 years of age
* Has one of the following risk factors:
* Severe aplastic anemia (SAA)
* Myelodysplastic features
* Karnofsky performance status ≥ 70% if ≥ 16 years of age or Lansky play score

≥ 50% for patients \<16 years of age
* Adequate pulmonary, cardiac and liver function
* Voluntary written consent (minor assent if appropriate) prior to the performance of any study related procedures not part of standard medical care


* an HLA-A, B, DRB1 matched sibling donor (matched sibling)
* an HLA-A, B, DRB1 matched related donor (other than sibling)
* a related donor mismatched at 1 HLA-A, B, C and DRB1 antigen
* 7-8/8 HLA-A,B,C,DRB1 allele matched unrelated donor per current institutional guidelines Patients and donors are typed for HLA-A and B using serological or molecular techniques and for DRB1 using high resolution molecular typing. If a donor has been selected on the basis of HLA-A, B, C and DRB1 typing as above, preference will be made for donors matched at the HLA-C locus.
* Body weight of at least 40 kilograms and at least 12 years of age
* Willing and able to undergo mobilized peripheral blood apheresis
* In general good health as determined by the medical provider
* Adequate organ function defined as:

* Hematologic: hemoglobin, WBC, platelet within 10% of upper and lower limit of normal range of test (gender based for hemoglobin)
* Hepatic: ALT \< 2 x upper limit of normal
* Renal: serum creatinine \< 1.8 mg/dl
* Performance of a donor infectious disease screen panel including CMV Antibody, Hepatitis B Surface Antigen, Hepatitis B Core Antibody, Hepatitis C Antibody, HIV 1/2 Antibody, HTLVA 1/2 Antibody, Treponema, and Trypanosoma Cruzi (T. Cruzi) plus HBV, HCV, WNV, HIV by nucleic acid testing (NAT); and screening for evidence of and risks factors for infection with Zika virus, or per current standard institutional donor screen - must be negative for HIV and active hepatitis B
* Not pregnant - females of childbearing potential must have a negative pregnancy test within 7 days of mobilization start
* Voluntary written consent (parent/guardian and minor assent, if \< 18 years) prior to the performance of any research related procedure

Exclusion Criteria

* Pregnant or breastfeeding as the treatment used in this study are Pregnancy Category D. Females of childbearing potential must have a negative pregnancy test (serum or urine) within 14 days of study registration
* Active, uncontrolled infection within 1 week prior to starting study therapy
* Malignant solid tumor cancer within previous 2 years
Maximum Eligible Age

65 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

Masonic Cancer Center, University of Minnesota

OTHER

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Margaret MacMillan, MD, Msc, FRCPC

Role: PRINCIPAL_INVESTIGATOR

Masonic Cancer Center, University of Minnesota

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Masonic Cancer Center at University of Minnesota

Minneapolis, Minnesota, United States

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

United States

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Margaret MacMillan, MD, Msc, FRCPC

Role: CONTACT

612-626-2961

Facility Contacts

Find local site contact details for specific facilities participating in the trial.

Lisa Burke, RN

Role: primary

612-273-8482

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

MT2017-17

Identifier Type: OTHER

Identifier Source: secondary_id

2016LS161

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.