Term
| What are factors associated with improved survival in SCID? |
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Definition
Younger age (<3.5mo for SCID, >5y of CGD and WAS)
SCID phenotype with absent NK cells
HLA-identical stem cell donor
No pre-transplant conditioning for SCID
Larger dose of CD34+ stem cells, lower dose of mature T cells
Absence of viral or fungal infection at time of transplant |
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Term
| What is the difference between partial chimera and full chimera of donor in WAS transplant? |
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Definition
| Partial chimera overcomes the defect in the WASP gene but full is needed for resolution of autoimmune manifestations |
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Term
| When can parental donors be used in stem cell transplant? |
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Definition
| T lymphocyte depleted haploidentical related (parental) donors can be used in pt with no innate T lymphocyte function such as SCID |
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Term
| What is the target dose of CD34+ cells for stem cell transplant? |
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Definition
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Term
| What is the time to engraftment and function for T cells, B cells, neutrophils and NK cells? |
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Definition
| T cells 3-4mo (CD8 before CD4 therefore CD4:CD8 <1), B cells 2-3y for function, innate/neutrophils weeks, NK cells months |
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Term
| Are live vaccines contraindicated if history of SCID s/p transplant? |
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Definition
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Term
When are live vaccines contraindicated with oral steroid use?
What about IVIG? |
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Definition
If more than 2mg/kg or 20mg/day for 2 weeks or more, delay for one month after stopping
If IVIG given wait 8-11mo prior to giving live vaccines (if live vaccines given wait 2 weeks prior to giving IVIG) |
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