[CIS PIDD] [cis-pidd] ADA-SCID

[Sullivan, Kathleen]

Hello all!
We have a 3m old ADA SCID baby.  She presented as a typical SCID with a viral pneumonitis.  Because of some custody issues, she was not an instant BMT candidate and so was placed on PEG-ADA.  A month into the PEG-ADA, she developed what looks like GVHD.  She has a desquamative rash, slight bump in her LFTs and increasing numbers of CD4CD45RO cells.  We will get a chimerism study but it prompts me to ask- could this be a delayed maternal engagement and the maternal cells were in hibernation until we metabolically corrected the baby?

Thoughts?

Kate Sullivan
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