[CIS PIDD] [cis-pidd] RAG1 SCID with loss of donor chimerism in myeloid lineage

[CIS-PIDD]

Dear all,
We follow 2 Amish  RAG1 SCID patients who have undergone fully matched related HCT and have shown loss of chimerism in the myeloid lineage over time.
These patients are clinically doing well and have 80-100% donor chimerism in CD3 T cells.
One patient is 5 yrs. since HCT and the other one is 5 months out.
Both of our patients had presented with Omenn's syndrome and received conditioning with Flu/Mel /Campath( day -8/9 to -6/7)  with Tac/Pred as GVHD prophylaxis.

We were intrigued and wanted to ask if a similar phenomenon was seen in other RAG1 SCID patients.

Thanks,

Avni Joshi
Mayo Clinic




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