RecruitingPhase 1NCT05241444

CD4^LVFOXP3 in Participants With IPEX

Phase 1 Study of Autologous CD4^LVFOXP3 in Participants With Immune Dysregulation Polyendocrinopathy Enteropathy X-linked (IPEX) Syndrome


Sponsor

Bacchetta, Rosa, MD

Enrollment

30 participants

Start Date

Mar 22, 2022

Study Type

INTERVENTIONAL

Conditions

Summary

This first-in-human, Phase 1 clinical trial will test the feasibility of the manufacturing and the safety of the administration of CD4\^LVFOXP3 in up to 30 evaluable human participants with IPEX and evaluate the impact of the CD4\^LVFOXP3 infusion on the disease.


Eligibility

Sex: MALEMin Age: 4 MonthsMax Age: 35 Years

Inclusion Criteria9

  • Body weight greater than 8 kg, unless assessed as able to tolerate leukapheresis
  • FOXP3 gene mutation
  • Medical history of progressive symptoms of IPEX with persistency of some symptoms and/or signs requiring immune suppressive medication. The participant may or may not be on immunosuppression at time of starting the study.
  • Uncontrolled IPEX disease but unable to tolerate immune suppressive medication
  • Recurrent IPEX symptoms, requiring immune suppressive medications, in participants who have had prior allogeneic (allo) blood stem cell transplantation (HSCT).
  • ≥ 50% Performance rating on Lansky/Karnofsky Scale
  • Organ and marrow function within acceptable levels of function
  • Absence of ongoing infections
  • Must be able to consent if an adult

Exclusion Criteria8

  • Medical instability
  • Less than 6 months life expectancy
  • Inability to meet limits for steroid dosing
  • Eligible for an HLA matched sibling or matched unrelated donor blood stem cell transplant, and be willing to undergo transplant.
  • Unrelated or comorbid disease
  • Allergy to any study medication, product, or intervention
  • Currently receiving another experimental treatment
  • History of malignancy, unless disease free for at least 2 years, with the exception of non melanoma skin cancer or carcinoma in situ

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Interventions

BIOLOGICALCD4^LVFOXP3

Infusion of autologous CD4+ T cells that have undergone lentiviral-mediated gene transfer of: i) healthy human FOXP3 gene leading to persistent high FOXP3 expression and acquisition of Treg-like cell function; and ii) human CD271 surface marker gene that allows tracking and quantification of the CD4\^LVFOXP3 in the blood.


Locations(1)

Lucile Packard Children's Hospital

Palo Alto, California, United States

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NCT05241444


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