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Phase 2 N=17 Treatment

MT2015-20: Biochemical Correction of Severe EB by Allo HSCT and Serial Donor MSCs

Epidermolysis Bullosa

Enrolled (actual)
17
Serious AEs
17.7%
Results posted
Sep 2024
Primary outcome: Primary: Event-free Survival — 4; 2; 1 Count of Participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Thymoglobulin (Drug); Cyclophosphamide (Drug); Fludarabine (Drug); Total Body Irradiation (Radiation); Bone marrow infusion (Procedure); Tacrolimus (Drug); Mycophenolate Mofetil (Drug); Donor mesenchymal stem cell infusions (Biological); Busulfan (Drug)
Age
Pediatric, Adult
Sex
All
Sponsor
Masonic Cancer Center, University of Minnesota
Primary completion
Nov 2022

Outcome Measures

OutcomeResultp-value
PRIMARY
Event-free Survival
4; 2; 1
SECONDARY
Change of a Patient's iscorEB
-10; -24; 110
SECONDARY
Transplant-related Mortality
0; 0; 0
SECONDARY
Average Change in Quality of Life
10; 0
SECONDARY
Average Change in Quality of Life
10; 0
SECONDARY
Lymphoid Chimerism
100; 60; NA; 99; 78; NA
SECONDARY
Myeloid Chimerism
76; 95; 23; 32; 96; 0
SECONDARY
Average Change of a Patient's iscorEB
9; -2

Summary

This is a single-institution, phase II study to determine the event-free survival at 1 year post allogeneic transplant and serial mesenchymal stem cell (MSC) infusions from a related donor (HLA identical, mismatched or haploidentical) or matched unrelated donor for the biochemical correction of severe epidermolysis bullosa (EB).

Eligibility Criteria

Inclusion Criteria

  • Diagnosis of severe form of EB characterized by collagen, laminin, integrin, keratin or plakin deficiency (by immunofluorescence staining with protein specific antibodies or Western blotting and by mutation analysis).
  • Adequate organ function within 4 weeks of study registration defined as:
  • Renal: glomerular filtration rate within normal range for age
  • Hepatic: Hepatic: bilirubin, AST/ALT, ALP < 5 x upper limit of normal
  • Pulmonary: adequate pulmonary function in the opinion of the enrolling investigator
  • Cardiac: left ventricular ejection fraction ≥ 45%, normal EKG or approved by Cardiology for transplant
  • Sexually active participants must agree to use adequate birth control for the during the study period (from before the start of the preparative chemotherapy through 1 year post-transplant)
  • Available donor per section 5: targeted MFI < 1,000 (MFI exceeding 1000 must be approved by the PI and treatment team.)
  • Voluntary written consent - adult or parent (with information sheet for minors, if applicable) prior to any research related procedures or treatment

Exclusion Criteria

  • beta 3 laminin JEB mutants
  • Active untreated systemic infection at time of transplantation (including active infection with Aspergillus or other mold within 30 days)
  • History of HIV infection
  • Evidence of squamous cell carcinoma
  • Pregnant or breast feeding. Females of child-bearing potential must have a negative pregnancy test prior to study registration as the agents administered in this study are Pregnancy Category C and D.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02582775). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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