NCT05787574

Brief Summary

The researchers are doing this study to find out whether emapalumab or a combination of fludarabine and dexamethasone are effective in preparing people with a primary immune regulatory disorder (PIRD) and/or an autoinflammatory condition to receive a stem cell transplant. The researchers will look at how well the study treatments reduce inflammation and aid in the engraftment process (the process of donated stem cells traveling to the bone marrow, where they begin to make new immune cells. "Funding Source - FDA OOPD"

Trial Health

77
On Track

Trial Health Score

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

Enrollment
39

participants targeted

Target at P25-P50 for phase_2

Timeline
10mo left

Started Mar 2023

Typical duration for phase_2

Geographic Reach
1 country

12 active sites

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 Progress79%
Mar 2023Mar 2027

First Submitted

Initial submission to the registry

March 15, 2023

Completed
Same day until next milestone

Study Start

First participant enrolled

March 15, 2023

Completed
13 days until next milestone

First Posted

Study publicly available on registry

March 28, 2023

Completed
3.9 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

March 1, 2027

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

March 1, 2027

Last Updated

October 29, 2025

Status Verified

October 1, 2025

Enrollment Period

4 years

First QC Date

March 15, 2023

Last Update Submit

October 28, 2025

Conditions

Keywords

Stem Cell TransplantEmapalumabFludarabineDexamethasone23-040

Outcome Measures

Primary Outcomes (1)

  • Engraftment

    is defined as the first of three days of absolute neutrophil count \>500k/µL and the first of seven days of platelets \>20,000/µL in the absence of transfusional support.

    1 year

Secondary Outcomes (1)

  • Determine Overall Survival (OS)

    5 years

Study Arms (2)

Group A: Emapalumab (for isolated Interferongamma mediated disease)

EXPERIMENTAL

Participants in this group will receive emapalumab on Days -22 (22 days before the day of the stem cell transplant), -15, -8, and -1.

Drug: EmapalumabProcedure: Stem Cell Transplant

Group B: Fludarabine and Dexamethasone (for generalized autoinflammation)

EXPERIMENTAL

Participants in this group will receive fludarabine and dexamethasone for 5 days in a row on Days -22 through -18.

Drug: Fludarabine and DexamethasoneProcedure: Stem Cell Transplant

Interventions

Emapalumab on Days -22 (22 days before the day of the stem cell transplant), -15, -8, and -1.

Group A: Emapalumab (for isolated Interferongamma mediated disease)

Fludarabine and dexamethasone for 5 days in a row on Days -22 through -18.

Group B: Fludarabine and Dexamethasone (for generalized autoinflammation)

Participants in both groups will receive their standard-of-care stem cell transplant on Day 0.

Group A: Emapalumab (for isolated Interferongamma mediated disease)Group B: Fludarabine and Dexamethasone (for generalized autoinflammation)

Eligibility Criteria

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

You may qualify if:

  • Patients receiving first allo-HCT for the following immunologic conditions:
  • Primary Immune Regulatory Disorder with or without a genetic lesion as defined by the Primary Immune Deficiency Treatment Consortium (PIDTC)
  • Patients with autoinflammatory disorders evidenced by cytokine or inflammation assays with at least 1.5x ULN of measured cytokines and/or an elevated ferritin or ESR \> 2 ULN
  • Able to tolerate cytoreduction (based on adequate organ function as described below)
  • Adequate organ function is required, defined as follows:
  • Hepatic: Serum bilirubin ≤ 2 mg/dL, unless benign congenital hyperbilirubinemia. Patients with hyperbilirubinemia related to paroxysmal nocturnal hemoglobinuria or other hemolytic disorders related to their PIRD diagnosis are eligible.
  • Hepatic: AST, ALT, and alkaline phosphatase \< 2.5 times the upper limit of normal unless thought to be disease-related. Investigator will need to perform clinically indicated evaluations to assess if disease related or intrinsic liver disease. Additional testing may be done if clinically indicated, after the pre-transplant immune prophase and prior to start of conditioning as this will provide additional data to confirm disease related versus intrinsic liver dysfunction.
  • Renal: serum creatinine \<1.5x normal for age. If serum creatinine is outside the normal range, then CrCl \> 50 mL/min/1.73m2 (calculated or estimated) or GFR (mL/min/1.72m2) \>30% of predicted normal for age.
  • Normal GFR by Age
  • Cardiac: LVEF ≥ 50% by MUGA or resting echocardiogram.
  • Pulmonary: Pulmonary function testing (FEV1 and corrected DLCO) ≥ 50% predicted (pediatric patients unable to complete PFTs will need oxygen saturation as recorded by pulse oximetry of ≥92% on room air).
  • Adequate performance status:
  • Age ≥ 16 years: ECOG ≤ 1 or Karnofsky 70%
  • Age \< 16 years: Lansky 70%
  • Each patient must be willing to participate as a research subject and must sign an informed consent form or legal guardian with assent as appropriate.

You may not qualify if:

  • Uncontrolled infection at the time of enrollment.
  • Patients who have undergone previous allo-HCT.
  • Patient seropositivity for HIV I/II and/or HTLV I/II.
  • Females who are pregnant or breastfeeding.
  • Patients unwilling to use contraception during the study period.
  • Patient or parent or guardian unable to give informed consent or unable to comply with the treatment protocol including research tests.
  • Related Donors:
  • /8 or 7/8 HLA matched at A, B, C, and DRB1 loci, as tested by DNA analysis.
  • Haploidentical donors at A, B, C and DRB1 loci, as tested by DNA analysis
  • Unrelated Donors:
  • o 8/8 or 7/8 matched at A, B, C, and DRB1 loci, as tested by DNA analysis.
  • Able to provide informed consent for the donation process per institutional standards.
  • Meet standard criteria for donor collection (e.g. National Marrow Donor Program Guidelines or collecting center guidelines as approved by treating physician).

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (12)

University of California, San Francisco (Data collection only)

San Francisco, California, 94143, United States

RECRUITING

Children's Healthcare of Atlanta (Data Collection Only)

Atlanta, Georgia, 30322, United States

RECRUITING

Memorial Sloan Kettering Basking Ridge (Limited Protocol Activities)

Basking Ridge, New Jersey, 07920, United States

RECRUITING

Memorial Sloan Kettering Monmouth (Limited Protocol Activities)

Middletown, New Jersey, 07748, United States

RECRUITING

Memorial Sloan Kettering Bergen (Limited Protocol Activities)

Montvale, New Jersey, 07645, United States

RECRUITING

Memorial Sloan Kettering Suffolk - Commack (Limited Protocol Activities)

Commack, New York, 11725, United States

RECRUITING

Memorial Sloan Kettering Westchester (Limited Protocol Activities)

Harrison, New York, 10604, United States

RECRUITING

Memorial Sloan Kettering Cancer Center

New York, New York, 10065, United States

RECRUITING

Memorial Sloan Kettering Nassau (Limited Protocol Activities)

Rockville Centre, New York, 11553, United States

RECRUITING

Children's Hospital of Philadelphia (Data Collection Only)

Philadelphia, Pennsylvania, 19104, United States

RECRUITING

Texas Children's Hospital (Data Collection)

Houston, Texas, 77030, United States

RECRUITING

Children's Hospital of Wisconsin (Data Collection Only)

Milwaukee, Wisconsin, 53226, United States

RECRUITING

Related Links

MeSH Terms

Conditions

Immune System Diseases

Interventions

EmapalumabfludarabineDexamethasoneStem Cell Transplantation

Intervention Hierarchy (Ancestors)

PregnadienetriolsPregnadienesPregnanesSteroidsFused-Ring CompoundsPolycyclic CompoundsSteroids, FluorinatedCell TransplantationCell- and Tissue-Based TherapyBiological TherapyTherapeuticsTransplantationSurgical Procedures, Operative

Study Officials

  • Andromachi Scaradavou, MD

    Memorial Sloan Kettering Cancer Center

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Andromachi Scaradavou, MD

CONTACT

Roni Tamari, MD

CONTACT

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NON RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
PARALLEL
Model Details: This is a two-stage phase 2 study.
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

March 15, 2023

First Posted

March 28, 2023

Study Start

March 15, 2023

Primary Completion (Estimated)

March 1, 2027

Study Completion (Estimated)

March 1, 2027

Last Updated

October 29, 2025

Record last verified: 2025-10

Data Sharing

IPD Sharing
Will share

Memorial Sloan Kettering Cancer Center supports the international committee of medical journal editors (ICMJE) and the ethical obligation of responsible sharing of data from clinical trials. The protocol summary, a statistical summary, and informed consent form will be made available on clinicaltrials.gov when required as a condition of Federal awards, other agreements supporting the research and/or as otherwise required. Requests for deidentified individual participant data can be made beginning 12 months after publication and for up to 36 months post publication. Deidentified individual participant data reported in the manuscript will be shared under the terms of a Data Use Agreement and may only be used for approved proposals. Requests may be made to: crdatashare@mskcc.org.

Locations