This is an open-label, single arm study to evaluate the cryopreserved formulation of OTL-103 Gene Therapy. OTL-103 consists of autologous CD34+ hematopoietic stem cells in which the gene encoding for the Wiskott-Aldrich Syndrome is introduced by means of a third generation lentiviral vector.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
10
Autologous hematopoietic stem cells collected from mobilized peripheral blood transduced ex vivo with a lentiviral vector encoding the WAS cDNA
Children's Healthcare of Atlanta, Inc
Atlanta, Georgia, United States
Ospedale San Raffaele - Telethon Institute for Gene Therapy (OSR-TIGET)
Milan, Italy
Annualized rate of severe infections from 6 to 18 months after gene therapy compared with 1 year before gene therapy
Time frame: 18 months
Annualized rate of moderate and severe bleeding episodes up to 1 year after gene therapy compared with 1 year before gene therapy
Time frame: 12 months
Evaluation of the overall survival
Time frame: 36 months
Number of patients with Vector copy number (VCN)/cell > 0.1 measured in peripheral blood-derived CD3+ cells
Time frame: 2 years
Percentage of WAS protein expression increased from pre-treatment levels in lymphocytes
Time frame: 2 years
Percentage of WAS protein expression increased from pre-treatment levels in platelets
Time frame: 2 years
Number of participants with successful engraftment of OTL-103
Engraftment of of OTL-103 is measured by hematological reconstitution of an absolute neutrophil count \> 500 cell/ul
Time frame: 6 months
The number of subjects presenting with malignancies or abnormal clonal proliferation
Time frame: 2 years
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