NCT04558736

Brief Summary

This study is a prospective, single center phase II clinical trial in which patients with Severe Aplastic Anemia (SAA) ) will receive a haploidentical transplantation. The purpose of this study is to learn more about newer methods of transplanting blood forming cells donated by a family member that is not fully matched to the patient. This includes studying the effects of the chemotherapy, radiation, the transplanted cell product and additional white blood cell (lymphocyte) infusions on the patient's body, disease and overall survival. The primary objective is to assess the rate of engraftment at 30 days and overall survival (OS) and event free survival (EFS) at 1 year post-hematopoietic cell transplantation (HCT). Primary Objectives

  • To estimate the rate of engraftment at 30 days after TCR αβ+ T-cell-depleted graft infusion in patients receiving a single dose of post graft infusion cyclophosphamide.
  • To estimate the overall survival and event free survival at 1-year post transplantation. Secondary Objectives
  • To calculate the incidence of acute and chronic GVHD after HCT.
  • To calculate the rate of secondary graft rejection at 1-year post transplantation
  • To calculate the cumulative incidence of viral reactivation (CMV, EBV and adenovirus).
  • To describe the immune reconstitution after TCR αβ+ T-cell-depleted graft infusion at 1 month, 3 months, 6 months, 9 months, and 1 year. Exploratory Objectives
  • To longitudinally assess the phenotype and epigenetic profile of T-cells in SAA patients receiving HCT for SAA.
  • To assess the phenotype and epigenetic profile of T-cells in DLI administered to SAA patients post HCT.
  • To longitudinally assess CD8 T cell differentiation status in SAA patients using an epigenetic atlas of human CD8 T cell differentiation.
  • To examine the effector functions and proliferative capacity of CD8 T cells isolated from SAA patients before and after DLI.
  • Quantify donor derived Treg cells at different time points in patients received HCT.
  • Determine Treg activation status at different stages after HCT.
  • Are specific features of the DLI product associated with particular immune repertoire profiles post-transplant?
  • How does the diversity and functional profile of the DLI product alter the response to pathogens in the recipient?
  • Do baseline features of the recipient's innate and adaptive immune cells correlate with post-transplant immune repertoires and response profiles?

Trial Health

77
On Track

Trial Health Score

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

Enrollment
21

participants targeted

Target at below P25 for phase_2

Timeline
51mo left

Started Jan 2021

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 Progress56%
Jan 2021Jul 2030

First Submitted

Initial submission to the registry

September 9, 2020

Completed
13 days until next milestone

First Posted

Study publicly available on registry

September 22, 2020

Completed
4 months until next milestone

Study Start

First participant enrolled

January 21, 2021

Completed
8.4 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

July 1, 2029

Expected
1 year until next milestone

Study Completion

Last participant's last visit for all outcomes

July 1, 2030

Last Updated

July 4, 2025

Status Verified

July 1, 2025

Enrollment Period

8.4 years

First QC Date

September 9, 2020

Last Update Submit

July 3, 2025

Conditions

Keywords

Haploidentical Transplant

Outcome Measures

Primary Outcomes (2)

  • Engraftment

    Rate of patients engrafting at day 30 after TCR αβ+ T-cell-depleted graft infusion in patients receiving a single dose of post graft infusion cyclophosphamide.

    30 days

  • Overall and event free survival

    Rate of overall survival and event free survival at 1-year post transplantation.

    1 year

Secondary Outcomes (4)

  • Graft vs host disease

    1 year

  • Graft rejection

    1 year

  • Viral reactivation

    1 year

  • Immune reconstitution

    1 year

Study Arms (1)

Haploidentical HCT

EXPERIMENTAL

To assess the safety and efficacy of haploidentical donor transplantation for patients with severe aplastic anemia who lack an available HLA-matched donor. The goal of this study is to develop a novel, reduced-toxicity, post-transplant pharmacologic immunosuppression (GVHD prophylaxis)- free, highly tolerogenic haploidentical transplant regimen that is associated with few post- transplant complications or late toxicities and is available promptly to all patients, irrespective of matched donor availability. Cells for infusion are prepared using the CliniMACS System.

Drug: Anti-Thymocyte Globulin (Rabbit)Drug: FludarabineDrug: CyclophosphamideDrug: MesnaDrug: G-CSFRadiation: Total Lymphoid Irradiation (TLI)Device: CliniMACSBiological: HPC, A InfusionBiological: CD45RA-depleted DLI

Interventions

Given intravenously (IV)

Also known as: thymoglobulin, rabbit ATG
Haploidentical HCT

Given intravenously (IV)

Also known as: Fludara Flu Dara
Haploidentical HCT

Given intravenously (IV)

Also known as: CYTOXAN
Haploidentical HCT
MesnaDRUG

Given intravenously (IV)

Also known as: MESNEX
Haploidentical HCT
G-CSFDRUG

Filgrastim is a human granulocyte colony-stimulating factor (G-CSF), produced by recombinant DNA technology. Dosage and Route of Administration: 5mcg/kg subcutaneous or intravenous daily until ANC \>2000 for 2 consecutive days, or as clinically indicated

Also known as: Neupogen, Filgrastim
Haploidentical HCT

TLI will be given at 800 cGy total dose in 4 fractions.

Also known as: TLI
Haploidentical HCT
CliniMACSDEVICE

The mechanism of action of the CliniMACS Cell Selection System is based on magnetic-activated cell sorting (MACS). The CliniMACS device is a powerful tool for the isolation of many cell types from heterogeneous cell mixtures, (e.g. apheresis products). These can then be separated in a magnetic field using an immunomagnetic label specific for the cell type of interest, such as CD3+ human T cells.

Also known as: Cell Selection System
Haploidentical HCT
HPC, A InfusionBIOLOGICAL

Given intravenously Day 0-HPC, A Infusion (TCR αβ+/CD19+-depleted graft)

Haploidentical HCT

CD45RA-depleted DLI will be given at least ONE week after engraftment

Haploidentical HCT

Eligibility Criteria

AgeUp to 21 Years
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64)

You may qualify if:

  • Age less than or equal to 21 years at time of enrollment.
  • Confirmed diagnosis of SAA or a single lineage cytopenia
  • (a) SAA or single lineage cytopenia will be defined as follows:
  • i. Bone marrow cellularity \< 25% or hypocellular marrow for age, AND
  • ii. One or more of the following (in peripheral blood): (i) Neutrophils \< 0.5 x10\^9/L (ii) Platelets \< 20 x10\^9/L, or platelet transfusion dependence (iii) Hemoglobin \<8g/dL, or red blood cell transfusion dependence
  • Does not have a suitable HLA-matched sibling donor (MSD) or volunteer 10/10 HLA-matched unrelated donor (MUD) available in the necessary time for progenitor cell donation.
  • Failed at least one trial of immunosuppressive therapy (IST) by being refractory (persistence of severe cytopenias and fulfillment of SAA disease criteria at least 3 months after initial IST) or having relapsed (initial improvement of cytopenias after first-line IST but then a later return to fulfillment of SAA disease criteria when IST is decreased or ceased). IST could have included ATG based regimens, calcineurin inhibitors and/or other higher dose therapy directed at the treatment of primary SAA. Patients with very severe aplastic anemia who are likely not to benefit from IST do not need to have failed a trial of IST and can proceed directly to HCT if they meet the rest of the criteria.
  • Has a suitable single haplotype matched (≥ 3 of 6) family member donor.
  • Patient and/or legal guardian must sign informed consent for HCT.
  • Adequate organ function defined as:
  • Left ventricular ejection fraction \> 40% or shortening fraction ≥ 25%.
  • Creatinine clearance (CrCl) or glomerular filtration rate (GFR) ≥ 50 ml/ min/1.73m2.
  • Forced vital capacity (FVC) ≥ 50% of predicted value; or pulse oximetry
  • ≥ 92% on room air if patient is unable to perform pulmonary function testing.
  • Karnofsky or Lansky (age-dependent) performance score ≥ 50.
  • +3 more criteria

You may not qualify if:

  • Diagnosis of Fanconi anemia. Fanconi anemia must be excluded by diepoxybutane (DEB) or equivalent testing.
  • Known clinical or genetic diagnosis of dyskeratosis congenita
  • Clonal cytogenetic abnormalities consistent with pre-myelodysplastic syndrome (pre- MDS) or MDS on marrow examination (e.g. Monosomy 7).
  • Diagnosis of myelodysplastic syndrome (MDS).
  • Presence of anti-donor HLA antibodies (positive anti-donor HLA antibody is defined as a positive cross-match test of any titer by complement- dependent cytotoxicity or flow cytometric testing or the presence of anti- donor HLA antibody to the high expression loci HLA-A, B, C, DRB1, or DPB1 with mean fluorescence intensity (MFI) \> 1000 by solid phase immunoassay).
  • Prior allogeneic hematopoietic cell transplant.
  • Prior solid organ transplant.
  • Known life-threatening reaction (i.e., anaphylaxis) to ATG that would prohibit use for the patient.
  • Uncontrolled bacterial, viral, or fungal infection at the time of enrollment. Uncontrolled is defined as progression or no clinical improvement on appropriate medical treatment.
  • Female patients who are pregnant (per institutional practice) or breast- feeding.
  • Prior malignancies except resected basal cell carcinoma or treated cervical carcinoma in situ. Cancer treated with curative intent \> 5 years previously will be allowed. Cancer treated with curative intent ≤ 5 years previously will not be allowed unless approved by the PI.
  • Alemtuzumab or ATG within 2 weeks of enrollment.
  • At least single haplotype matched (≥ 3 of 6) family member.
  • At least 18 years of age.
  • HIV negative.
  • +5 more criteria

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

St. Jude Children's Research Hospital

Memphis, Tennessee, 38105, United States

RECRUITING

Related Links

MeSH Terms

Conditions

Anemia, AplasticBone Marrow Failure Disorders

Interventions

Antilymphocyte SerumthymoglobulinfludarabineCyclophosphamideMesnaGranulocyte Colony-Stimulating FactorFilgrastim

Condition Hierarchy (Ancestors)

AnemiaHematologic DiseasesHemic and Lymphatic DiseasesBone Marrow Diseases

Intervention Hierarchy (Ancestors)

Immune SeraAntibodiesImmunoglobulinsImmunoproteinsBlood ProteinsProteinsAmino Acids, Peptides, and ProteinsSerum GlobulinsGlobulinsBiological ProductsComplex MixturesPhosphoramide MustardsNitrogen Mustard CompoundsMustard CompoundsHydrocarbons, HalogenatedHydrocarbonsOrganic ChemicalsPhosphoramidesOrganophosphorus CompoundsAlkanesulfonatesAlkanesulfonic AcidsAlkanesHydrocarbons, AcyclicSulfhydryl CompoundsSulfur CompoundsSulfonic AcidsSulfur AcidsColony-Stimulating FactorsGlycoproteinsGlycoconjugatesCarbohydratesHematopoietic Cell Growth FactorsCytokinesIntercellular Signaling Peptides and ProteinsPeptidesBiological Factors

Study Officials

  • Amr Qudeimat, MD

    St. Jude Children's Research Hospital

    PRINCIPAL INVESTIGATOR
  • Akshay Sharma, MBBS

    St. Jude Children's Research Hospital

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NA
Masking
NONE
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

September 9, 2020

First Posted

September 22, 2020

Study Start

January 21, 2021

Primary Completion (Estimated)

July 1, 2029

Study Completion (Estimated)

July 1, 2030

Last Updated

July 4, 2025

Record last verified: 2025-07

Data Sharing

IPD Sharing
Will share

Individual participant de-identified datasets containing the variables analyzed in the published article will be made available (related to the study primary or secondary objectives contained in the publication). Supporting documents such as the protocol, statistical analyses plan, and informed consent are available through the CTG website for the specific study. Data used to generate the published article will be made available at the time of article publication. Investigators who seek access to individual level de-identified data will contact the computing team in the Department of Biostatistics (ClinTrialDataRequest@stjude.org) who will respond to the data request.

Shared Documents
STUDY PROTOCOL, SAP, ICF
Time Frame
Data will be made available at the time of article publication.
Access Criteria
Data will be provided to researchers following a formal request with the following information: full name of requestor, affiliation, data set requested, and timing of when data is needed. As an informational point, the lead statistician and study principal investigator will be informed that primary results datasets have been requested.

Locations