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Sickle Cell DiseasePhase 1NCT02867800

Abatacept for GVHD Prophylaxis After Hematopoietic Stem Cell Transplantation for Pediatric Sickle Cell Disease

A Phase 1 study testing Diphenhydramine, Acetaminophen and Methylprednisolone in people with Sickle Cell Disease and Graft Versus Host Disease, run by Monica Bhatia at 7 sites in United States.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

To assess the tolerability of the costimulation blocking agent abatacept (CTLA4-Ig) when added to the standard graft versus host disease (GVHD) prophylaxis regimen of a calcineurin inhibitor and methotrexate in patients receiving early alemtuzumab followed by fludarabine, thiotepa, melphalan, and alemtuzumab for… Read more about this study

Who is it for?
Ages 3 to 20 years
What is being tested?
Diphenhydramine, Acetaminophen and Methylprednisolone
Could I receive a placebo?
No, everyone receives an active treatment
How many people
24 participants
Where
7 sitesUnited States
Study dates
Jul 2016to 30 Dec 2023
Phase
Phase 1
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 3 to 20 years
  • All sexes
  • No healthy volunteers
  • Child, Adult

Who may be able to join

  • Patients with hemoglobin (Hgb) SS or SB0 thalassemia between the ages of 3 and 20.99 years who are at least 10 kg getting an human leukocyte antigen (HLA) matched bone marrow transplant, will be eligible if they are at increased risk for graft versus host disease (GVHD) and have severe SCD.
  • Patients falling into one of the following three groups will be considered to be at increased risk for GVHD:
  • Are between 10 and 20.99 years and receiving their transplant from an HLA matched related donor.
  • Are between 3 and 9.99 years and receiving their transplant from an HLA matched related donor who is at least 10 years.
  • Are between 3 and 20.99 years and receiving their transplant from an HLA matched unrelated donor. Donors must be matched at the A, B, C and DRB1 loci at the allele level.
  • Patients who meet one of the following criteria will qualify as having severe SCD:
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  • Previous clinical stroke, as evidenced by a neurological deficit lasting longer than 24 hours, which is accompanied by radiographic evidence of ischemic brain injury and cerebral vasculopathy.
  • Asymptomatic cerebrovascular disease, as evidenced by one the following:
  • Progressive silent cerebral infarction, as evidenced by serial MRI scans that demonstrate the development of a succession of lesions (at least two temporally discreet lesions, each measuring at least 3 mm in greatest dimension on the most recent scan) or the enlargement of a single lesion, initially measuring at least 3 mm). Lesions must be visible on T2-weighted MRI sequences.
  • Cerebral arteriopathy, as evidenced by abnormal transcranial doppler (TCD) testing (confirmed elevated velocities in any single vessel of time-averaged maximum mean velocities (TAMMV) > 200 cm/sec for non-imaging TCD) or by significant vasculopathy on magnetic resonance angiogram (MRA) (greater than 50% stenosis of > 2 arterial segments or complete occlusion of any single arterial segment).
  • Frequent ( ≥ 3 per year for preceding 2 years) painful vaso-occlusive episodes (defined as episode lasting ≥ 4 hours and requiring hospitalization or outpatient treatment with parenteral opioids). If patient is on hydroxurea and its use has been associated with a decrease in the frequency of episodes, the frequency should be gauged from the 2 years prior to the start of this drug.
  • Recurrent ( ≥ 3 in lifetime) acute chest syndrome events which have necessitated erythrocyte transfusion therapy.
  • Any combination of ≥ 3 acute chest syndrome episodes and vaso-occlusive pain episodes (defined as above) yearly for 3 years. If patient is on hydroxurea and its use has been associated with a decrease in the frequency of episodes, the frequency should be gauged from the 3 years prior to the start of this drug.
  • All patients and/or their parents or legal guardians must sign a written informed consent. Assent, when appropriate, will be obtained according to institutional guidelines.
  • Must have been evaluated and adequately counseled regarding treatment options for severe SCD by a pediatric hematologist.
  • Because of the elective and non-urgent nature of hematopoietic stem cell transplantation (HSCT) for SCD, it is important that all patients and families be counseled regarding fertility preservation measures available to them. All patients and/or their parents or legal guardians must indicate on the consent and assent forms that they have received this counseling.

Who cannot join

  • Bridging (portal to portal) fibrosis or cirrhosis of the liver.
  • Parenchymal lung disease stemming from SCD or other process defined as a diffusing capacity of the lungs for carbon monoxide (DLCO) (corrected for hemoglobin) or forced vital capacity of less than 45% of predicted. Children unable to perform pulmonary function testing will be excluded if they require daytime oxygen supplementation.
  • Renal dysfunction with an estimated glomerular filtration rate (GFR) < 50% of predicted normal for age.
  • Cardiac dysfunction with shortening fraction < 25%.
  • Neurologic impairment other than hemiplegia, defined as full-scale IQ ≤70, quadriplegia or paraplegia, inability to ambulate, or any impairment resulting in decline of Lansky performance score to < 70%.
  • Clinical stroke within 6 months of anticipated transplant.
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  • Karnofsky or Lansky functional performance score < 70%.
  • Patient is human immunodeficiency virus (HIV) infected.
  • Donor is HIV infected.
  • Donor has Hgb SS, SC or SB0 thalassemia.
  • Patient with unspecified chronic toxicity serious enough to detrimentally affect the patient's capacity to tolerate bone marrow transplantation.
  • Patient or patient's guardian(s) unable to understand the nature and risks inherent in the bone marrow transplant (BMT) process.
  • History of lack of compliance with medical care that would jeopardize transplant course.
  • Donor who for psychological, physiologic, or medical reasons is unable to tolerate a bone marrow harvest or receive general anesthesia.
  • Active viral, bacterial, fungal or protozoal infection.
  • Donor is pregnant.
  • Patient is pregnant.

Trial locations and contacts

7 sites in 1 country.

7 sites in 1 country

United States7 sites
  • Children's Healthcare of Atlanta

    Atlanta, Georgia, 30322

  • Floating Hospital for Children at Tufts Medical Center

    Boston, Massachusetts, 02111

  • North Carolina Cancer Hospital

    Chapel Hill, North Carolina, 27514

  • Ann & Robert H. Lurie Children's Hospital of Chicago

    Chicago, Illinois, 60611

  • Nationwide Children's Hospital

    Columbus, Ohio, 43205

  • Columbia University Irving Medical Center

    New York, New York, 10032

  • Children's National Medical Center

    Washington D.C., District of Columbia, 20010

Other trials in United States

About this study

Official title: Abatacept for Graft Versus Host Disease Prophylaxis After Hematopoietic Stem Cell Transplantation for Pediatric Sickle Cell Disease: a Sickle Transplant Alliance for Research Trial

To assess the tolerability of the costimulation blocking agent abatacept (CTLA4-Ig) when added to the standard graft versus host disease (GVHD) prophylaxis regimen of a calcineurin inhibitor and methotrexate in patients receiving early alemtuzumab followed by fludarabine, thiotepa, melphalan, and alemtuzumab for conditioning.

Read the detailed description

Outcomes of hematopoietic stem cell transplantation (HSCT) for children and adolescents with sickle cell disease (SCD) have improved. Graft versus host disease (GVHD), however, remains a barrier to success. GVHD accounts for most of the transplant related mortality and much of the morbidity in this setting-in part through the injury it directly causes and in part through the deleterious effects of steroids and the other immunosuppressive agents used to prevent and treat it. The results of pre-clinical studies and a phase I clinical study in patients with hematologic malignancies suggest that the costimulation blocking agent CTLA4-Ig may hold promise an agent for GVHD prophylaxis. In the present trial, the investigators are assessing the tolerability of adding abatacept to standard GVHD prophylaxis-a calcineurin inhibitor and methotrexate-in pediatric SCD patients receiving early alemtuzumab (completed by day -18) followed by fludarabine, thiotepa, and melphalan for conditioning. This trial will provide the foundation for subsequent trials designed to test the long-term hypothesis that abatacept is a safe, steroid-sparing effective adjunct to standard GVHD prophylaxis in this setting.

Being tested
Diphenhydramine, Acetaminophen, Methylprednisolone, Meperidine, Alemtuzumab
Keywords
Sickle Cell Disease, Graft Versus Host Disease, Abatacept, Hematopoietic Stem Cell Transplantation
Medical subject headings
Anemia, Sickle Cell, Graft vs Host Disease

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • DiphenhydramineDrug

    Also called Benadryl

    (Standard of Care) Premedication

    Diphenhydramine: 1 mg/kg IV or PO q 8 hours (maximum=50 mg)

  • AcetaminophenDrug

    Also called Tylenol

    (Standard of Care) Premedication

    Acetaminophen: 10-15 mg/kg PO q 6 hours (maximum=1000 mg)

  • MethylprednisoloneDrug

    Also called Medrol

    (Standard of Care) Premedication

    Methylprednisolone: 0.25-0.5 mg/kg IV q 6 hours

  • MeperidineDrug

    Also called Demerol

    (Standard of Care) Premedication

    Use as needed (PRN) Meperidine: 0.5 mg/kg IV q 4-6 hours (for rigors)

  • AlemtuzumabDrug

    Also called Lemtrada

    (Standard of Care) Immunosuppression

    Alemtuzumab:

    A test dose of alemtuzumab, 3 mg, should be administered IV over 2 hours the first day. If the test dose is tolerated, administration of three treatment doses should begin within 24 hours. The three treatment doses should be administered on consecutive days. 10 mg/m2 should be given the first day, 15 mg/m2 the second and 20 mg/m2 the third.

  • ThymoglobulinDrug

    Also called Genzyme

    (Standard of Care) Immunosuppression

    Thymoglobulin: A 4 mg/kg dose of anti-thymocyte globulin should be administered in place of each alemtuzumab dose not completed.

  • FludarabineDrug

    Also called Fludara

    (Standard Conditioning Regimen)

    Fludarabine should be administered 30 mg/m2 IV daily for five days. It should be infused over 30 to 60 minutes.

  • MelphalanDrug

    Also called Alkeran

    (Standard Conditioning Regimen)

    Melphalan should be administered 140 mg/m2 IV 3 days before marrow infusion. It should be infused within 60 minutes of preparation and over a maximum of 30 minutes. It should be infused immediately after the fludarabine infusion is complete.

  • ThiotepaDrug

    Also called Thioplex

    (Standard Conditioning Regimen)

    Thiotepa should be administered 8 mg/kg IV 3 days before marrow infusion. It should be infused immediately after the fludarabine infusion is complete. The thiotepa should be infused over one hour.

  • CyclosporineDrug

    Also called Neoral

    (Standard GVHD Prophylaxis) Calcineurin inhibitor

    Cyclosporine: Administration will commence no later than at least 36 hours before marrow infusion; Cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml.

  • TacrolimusDrug

    Also called Protopic

    (Standard GVHD Prophylaxis) Calcineurin inhibitor

    Tacrolimus: Administration will commence no later than at least 36 hours before marrow infusion; Tacrolimus doses will be adjusted to maintain a level of 8-15 ng/ml.

  • MethotrexateDrug

    Also called Trexall

    (Standard GVHD Prophylaxis)

    Methotrexate will be given at a dose of 15 mg/m2 IV on day 1 and a dose of 10 mg/m2 IV on days 3, 6 and 11. Dosing shall be based on actual weight.

  • AbataceptDrug

    Also called CTLA4-Ig, Orencia

    (Investigational)

    Abatacept will be administered intravenously at a dose of 10 mg/kg based on actual weight with a maximum of 750 mg. In cases where the calculated dose is less than or equal to 110% of a simple multiple of a 250 mg vial: 250 mg (275mg), 500 mg (550 mg) or 750 mg (825 mg), the dose may be rounded down to the nearest multiple. No rounding up of the abatacept dose is permitted.

  • Marrow infusionProcedure or surgery

    Also called stem cell transplant

    (Standard)

    A procedure that infuses healthy cells, called stem cells, into your body to replace damaged or diseased bone marrow. A bone marrow transplant may also be used to treat certain types of cancer

  • SirolimusDrug

    Also called Rapamune

    (Standard GVHD Prophylaxis)

    Calcineurin inhibitor (cyclosporine or tacrolimus or with permission of the sponsor: sirolimus or mycophenolate mofetil (MMF)) administration will commence no later than day -2 (at least 36 hours before the stem cell infusion); cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml. This range assumes monitoring by mass spectrometry. If an immunoassay is used instead, the equivalent range for that immunoassay should be used.

  • Mycophenolate MofetilDrug

    Also called MMF

    (Standard GVHD Prophylaxis)

    Calcineurin inhibitor (cyclosporine or tacrolimus or with permission of the sponsor: sirolimus or mycophenolate mofetil (MMF)) administration will commence no later than day -2 (at least 36 hours before the stem cell infusion); cyclosporine doses will be adjusted to maintain a level of 150-300 ng/ml. This range assumes monitoring by mass spectrometry. If an immunoassay is used instead, the equivalent range for that immunoassay should be used.

Study groups in NCT02867800
Study groupRoleWhat they receive
Standard GVHD Prophylaxis + AbataceptExperimentalSubjects will receive * premedication (Diphenhydramine, Acetaminophen, Methylprednisolone; and Meperidine as needed) * immunosuppression (Alemtuzumab, or Thymoglobulin) * conditioning regimen (Fludarabine, Thiotepa, and Melphalan) * GVHD prophylaxis: calcineurin inhibitor (Cyclosporine,Tacrolimus, Sirolimus or Mycophenolate Mofetil with permission of the sponsor) and Methotrexate plus Abatacept on days -1, +5, +14 and +28, and a marrow infusion on day 0.
Receives: Drug: Diphenhydramine; Drug: Acetaminophen; Drug: Methylprednisolone; Drug: Meperidine; Drug: Alemtuzumab; Drug: Thymoglobulin; Drug: Fludarabine; Drug: Melphalan; Drug: Thiotepa; Drug: Cyclosporine; Drug: Tacrolimus; Drug: Methotrexate; Drug: Abatacept; Procedure: Marrow infusion; Drug: Sirolimus; Drug: Mycophenolate Mofetil

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Number of patients who tolerate abatacept

    Patients will be deemed to be evaluable for tolerability if they received all prescribed doses of abatacept.

    Abatacept will deemed to be tolerated, if no more than one dose is withheld per protocol stipulations, no death from an infection that occurs within 30 days of or develops post-transplant lymphoproliferative disease (PTLD) within 100 days of receiving the last prescribed dose of abatacept.

    Measured: Within 100 days of receiving the last prescribed dose of abatacept

Secondary outcomes (2)
  1. Bearman Scale Score of Regimen-Related Toxicity (RRT)

    RRT will be assessed according to Bearman Scale.

    Measured: Day 42 post-transplant

  2. Number of infections

    Infections will include viremia, posttransplant lymphoproliferative disease and immune reconstitution.

    Measured: Up to 180 days post transplant

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted16 Aug 2016 (estimated)
  2. Study startJul 2016
  3. Primary completion30 Dec 2021
  4. Study completion30 Dec 2023

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 1
Primary purpose
Treatment
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrolment
24 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT02867800
Sponsor's protocol ID
AAAQ2350
Status last verified
Jun 2024
First submitted
11 Aug 2016
Last updated
13 Jun 2024
Results posted
No
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
Individual data sharing
No

Questions about this trial

What is this clinical trial studying?

A Phase 1 study testing Diphenhydramine, Acetaminophen and Methylprednisolone in people with Sickle Cell Disease and Graft Versus Host Disease, run by Monica Bhatia at 7 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 16 inclusion and 17 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 13 Jun 2024. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 7 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 1 studies test a treatment in a small group of people for the first time or in a new way, mainly to check safety and find the right dose.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Number of patients who tolerate abatacept (time frame: Within 100 days of receiving the last prescribed dose of abatacept). It is what the study was designed to measure, not a finding.

Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 13 Jun 2024.

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