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Autologous hematopoietic stem cell gene therapy in RAG-deficient severe combined immunodeficiency: pre-phase study

Recruiting
Conditions
severe combined immunodeficiency
10021460
Registration Number
NL-OMON55430
Lead Sponsor
eids Universitair Medisch Centrum
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Recruiting
Sex
Not specified
Target Recruitment
8
Inclusion Criteria

Selection as stem cell donor for a family member;
- In case of stem cells from mobilized peripheral blood: completion of G-CSF
mobilization procedure for collection of CD34+ HSC from G-CSF mobilized
peripheral blood;
Age > 18 years;
Able to comprehend and give signed informed consent.

Exclusion Criteria

Any condition precluding stem cell donation
Pregnancy

Study & Design

Study Type
Observational invasive
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>Successful transduction of fresh CD34+ hematopoietic stem cells with a<br /><br>lentiviral SIN vector encoding codon-optimized human RAG1 or RAG2 cDNA<br /><br>(SIN-LV-RAG1 or RAG2 vector), defined as a vector copy number higher than 0.5.</p><br>
Secondary Outcome Measures
NameTimeMethod
<p>Total number and percentage of viable CD34+ HSC after thawing of cryopreserved<br /><br>transduced cells.<br /><br>Failure to meet release criteria as defined in IMPD.<br /><br>in vitro and/or in vivo functionality assays of transduced CD34+ HSC after<br /><br>thawing.</p><br>
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