Status:

NOT_YET_RECRUITING

Hematopoietic Stem Cell Transplantation Gene Therapy for Treatment of Severe Hemophilia A

Lead Sponsor:

Expression Therapeutics, LLC

Conditions:

Hemophilia A

Eligibility:

MALE

18+ years

Phase:

PHASE1

Brief Summary

This is a first-in-human, non-randomized, open label, single treatment, Phase 1 study in approximately 7 patients with severe hemophilia A. The study will evaluate gene therapy by transplantation of a...

Detailed Description

Eligible subjects will undergo CD34+ hematopoietic stem cell collection. These cells will be transduced ex vivo with CD68-ET3 lentiviral vector and subsequently, following a conditioning regimen of bu...

Eligibility Criteria

Inclusion

  • Able to provide informed consent for the protocol approved by the Institutional Review Board.
  • Male subjects who are \>= 18 years of age.
  • Diagnosis of severe hemophilia A (\<1 IU/dL factor VIII activity) based on one-stage coagulation assay.
  • Documented history of more than 150 days of factor VIII treatment.
  • Average of at least 4 bleeds requiring treatment per year over the prior three years, or at least 4 bleeds per year during the 3 years preceding the initiation of prophylaxis, or evidence of joint damage (knee, elbow or ankle) on physical or radiographic examination thought to be related to hemophilia.
  • Performance status (Karnofsky score) of at least 70.
  • Willingness to use effective barrier contraception or limit sexual intercourse to postmenopausal, surgically sterilized, or contraception-practicing partners, for 12 weeks (3 months) after transplantation.
  • Willing and able to comply with the requirements of the protocol.

Exclusion

  • History of spontaneous central nervous system bleeding within the last 5 years.
  • Significant functional deficits in major organs which would interfere with successful outcome following autologous stem cell transplant, the following guidelines will be utilized:
  • Cardiac: There should be no evidence of significant cardiac dysfunction (resting left ventricular ejection fraction of \< 50%) and no marked cardiomegaly. There should not be uncontrollable hypertension.
  • Renal: GFR \< 60 mL/min/1.73m2 per local institutional standard such as CKD-EPI creatinine equation or equivalent.
  • Hepatic: There should be no evidence of hepatic dysfunction which is defined as a serum total bilirubin of \> 1.5 mg/dL and AST/ALT \> 3X the upper limit of normal.
  • Hematologic: Absolute neutrophil counts (ANC) \<1000/ µL or platelets counts \< 150,000/µL.
  • Pulmonary function with a corrected carbon monoxide diffusing capacity (cDLCO) \< 50% predicted.
  • History of a fVIII inhibitor (\> 0.4 Bethesda Units/mL) including at least 2 measurements done at least a week apart or any single titer \> 5 BU/mL.
  • Subjects who have had prior cellular based therapy or gene editing/ gene therapy including a previous stem cell transplant.
  • Subjects with any evidence of active infection or any immunosuppressive disorder, including currently detectable HIV viral load
  • Subjects who are RPR, anti-HTLV-1 and II antibody, CMV PCR, VZV antibody and HSV PCR positive at screening.
  • Subjects who have allergic reactions or hypersensitivity to any of the drugs used in the study (i.e., anti-thymocyte globulin, plerixafor, G-CSF, busulfan, levetiracetam) or to the constituents of the investigational product formulation.
  • Evidence of hepatitis B active infection or chronic carrier based on a positive Hepatitis B DNA testing at screening.
  • Positive (detectable viral load per local institutional standard) for the presence of Hepatitis C virus (HCV). Subjects who are positive for anti-HCV antibody are eligible as long as they have a negative undetectable HCV viral load at screening.
  • Subjects diagnosed with any history of clinically relevant coagulation or bleeding disorder other than hemophilia A.
  • Use of medication(s) that can affect hemostasis (e.g. aspirin, ibuprofen and non-COX-2 selective non-steroid anti-inflammatory drugs).
  • Subjects with a history of a malignancy (except surgically resected non-melanoma skin cancer) or subjects with a family history of a known cancer syndrome in a first degree relative.
  • Planned surgery within 6 months of enrollment (other than study procedures).
  • Treatment with any live vaccines or systemic immunosuppressive agents, not including corticosteroids within 30 days before CD68-ET3-LV CD34+ infusion.
  • Treatment with any investigational product within 30 days or 5 half-lives of the investigational product (whichever is longer) prior to enrollment.
  • History of autoimmune disease (e.g., inflammatory bowel disease, systemic lupus erythematosus, vasculitis).
  • Concurrent enrollment in another clinical study, which might interfere with the requirements of this study or have the potential to impact the evaluation of safety and efficacy of CD68-ET3-LV CD34+- unless it is a non-interventional observational study.
  • Any condition in the opinion of the Study Investigators that will negatively impact the subject's ability to safely undergo an autologous stem cell transplant.
  • Any reason in the opinion of the Study Investigators that will negatively impact the subject's ability to complete the clinical trial per the trial protocol.

Key Trial Info

Start Date :

September 1 2024

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

August 1 2039

Estimated Enrollment :

7 Patients enrolled

Trial Details

Trial ID

NCT04418414

Start Date

September 1 2024

End Date

August 1 2039

Last Update

February 20 2024

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