NCT03579875

Brief Summary

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.

Trial Health

77
On Track

Trial Health Score

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

Enrollment
48

participants targeted

Target at P25-P50 for phase_2

Timeline
33mo left

Started Nov 2018

Longer than P75 for phase_2

Geographic Reach
1 country

1 active site

Status
recruiting

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

Study Progress74%
Nov 2018Jan 2029

First Submitted

Initial submission to the registry

May 25, 2018

Completed
2 months until next milestone

First Posted

Study publicly available on registry

July 9, 2018

Completed
4 months until next milestone

Study Start

First participant enrolled

November 13, 2018

Completed
8.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

January 1, 2027

Expected
2 years until next milestone

Study Completion

Last participant's last visit for all outcomes

January 5, 2029

Last Updated

January 30, 2026

Status Verified

January 1, 2026

Enrollment Period

8.1 years

First QC Date

May 25, 2018

Last Update Submit

January 29, 2026

Conditions

Outcome Measures

Primary Outcomes (1)

  • Grade II-IV acute graft versus host disease (GVHD)

    incidence of grade II-IV acute graft versus host disease (GVHD)

    Day 100

Secondary Outcomes (8)

  • Neutrophil engraftment

    Day 42

  • Platelet engraftment

    Day 42

  • Acute graft versus host disease (aGVHD)

    Day 100

  • Chronic graft versus host disease (cGVHD)

    1 Year after transplant

  • Regimen related toxicity

    30 Days after transplant

  • +3 more secondary outcomes

Study Arms (5)

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

EXPERIMENTAL

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

Drug: Total Body Irradiation (TBI) (Plan 1)Drug: Cyclophosphamide (CY) (Plan 1)Drug: Fludarabine (FLU)Drug: Methylprednisolone (MP)Device: Donor mobilized PBSC infusionDrug: G-CSFDrug: Rituximab

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

EXPERIMENTAL

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

Drug: Fludarabine (FLU)Drug: Methylprednisolone (MP)Device: Donor mobilized PBSC infusionDrug: G-CSFDrug: Cyclophosphamide (CY) (Plan 2)Drug: Rituximab

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

EXPERIMENTAL

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

Drug: Cyclophosphamide (CY) (Plan 1)Drug: Fludarabine (FLU)Drug: Methylprednisolone (MP)Drug: RituximabDrug: Busulfan

Treatment Plan 4: CY, FLU, and alemtuzumab

EXPERIMENTAL

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.

Drug: Cyclophosphamide (CY) (Plan 1)Drug: Fludarabine (FLU)Drug: Alemtuzumab

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

EXPERIMENTAL

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.

Drug: Cyclophosphamide (CY) (Plan 1)Drug: Fludarabine (FLU)Drug: AlemtuzumabDrug: Melphalan

Interventions

300 cGy with thymic shielding on day -6

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

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

Also known as: CY
Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 3: BU, Cy, FLU, MP and Rituximab in patients with Fanconi AnemiaTreatment Plan 4: CY, FLU, and alemtuzumabTreatment Plan 5: CY, FLU, melphalan (MEL), and alemtuzumab.

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

Also known as: FLU
Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 3: BU, Cy, FLU, MP and Rituximab in patients with Fanconi AnemiaTreatment Plan 4: CY, FLU, and alemtuzumabTreatment Plan 5: CY, FLU, melphalan (MEL), and alemtuzumab.

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

Also known as: MP
Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 3: BU, Cy, FLU, MP and Rituximab in patients with Fanconi Anemia

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

Also known as: PBSC
Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi Anemia
G-CSFDRUG

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)

Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi Anemia

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

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

200 mg/m2 IV once on day -1

Treatment Plan 1: TBI 300 , CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 2: CY, FLU, MP, Rituximab in patients with Fanconi AnemiaTreatment Plan 3: BU, Cy, FLU, MP and Rituximab in patients with Fanconi Anemia

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.

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

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

Treatment Plan 4: CY, FLU, and alemtuzumabTreatment Plan 5: CY, FLU, melphalan (MEL), and alemtuzumab.

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.

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

Eligibility Criteria

AgeUp to 65 Years
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • 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:
  • +6 more criteria

You may not qualify if:

  • 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
  • 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
  • /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
  • +2 more criteria

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Masonic Cancer Center at University of Minnesota

Minneapolis, Minnesota, 55455, United States

RECRUITING

MeSH Terms

Conditions

Fanconi AnemiaAnemia, AplasticMyelodysplastic SyndromesBone Marrow Failure DisordersDyskeratosis Congenita

Interventions

Whole-Body IrradiationCyclophosphamidefludarabineMethylprednisoloneGranulocyte Colony-Stimulating FactorRituximabBusulfanAlemtuzumabMelphalan

Condition Hierarchy (Ancestors)

Anemia, Hypoplastic, CongenitalAnemiaHematologic DiseasesHemic and Lymphatic DiseasesCongenital Bone Marrow Failure SyndromesBone Marrow DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesDNA Repair-Deficiency DisordersMetabolic DiseasesNutritional and Metabolic DiseasesSkin AbnormalitiesCongenital AbnormalitiesGenetic Diseases, X-LinkedSkin Diseases, GeneticSkin DiseasesSkin and Connective Tissue Diseases

Intervention Hierarchy (Ancestors)

RadiotherapyTherapeuticsInvestigative TechniquesPhosphoramide MustardsNitrogen Mustard CompoundsMustard CompoundsHydrocarbons, HalogenatedHydrocarbonsOrganic ChemicalsPhosphoramidesOrganophosphorus CompoundsPrednisolonePregnadienetriolsPregnadienesPregnanesSteroidsFused-Ring CompoundsPolycyclic CompoundsColony-Stimulating FactorsGlycoproteinsGlycoconjugatesCarbohydratesHematopoietic Cell Growth FactorsCytokinesIntercellular Signaling Peptides and ProteinsPeptidesAmino Acids, Peptides, and ProteinsProteinsBiological FactorsAntibodies, Monoclonal, Murine-DerivedAntibodies, MonoclonalAntibodiesImmunoglobulinsImmunoproteinsBlood ProteinsSerum GlobulinsGlobulinsButylene GlycolsGlycolsAlcoholsMesylatesAlkanesulfonatesAlkanesulfonic AcidsAlkanesHydrocarbons, AcyclicSulfonic AcidsSulfur AcidsSulfur CompoundsAntibodies, Monoclonal, HumanizedPhenylalanineAmino Acids, AromaticAmino Acids, CyclicAmino Acids

Study Officials

  • Margaret MacMillan, MD, Msc, FRCPC

    Masonic Cancer Center, University of Minnesota

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Margaret MacMillan, MD, Msc, FRCPC

CONTACT

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
PARALLEL
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

May 25, 2018

First Posted

July 9, 2018

Study Start

November 13, 2018

Primary Completion (Estimated)

January 1, 2027

Study Completion (Estimated)

January 5, 2029

Last Updated

January 30, 2026

Record last verified: 2026-01

Locations