[CIS-PAGID] non conditioned BMT in a SCIDs case

[Aly.Mageed at helendevoschildrens.org]

What does the group think of a 4YO with a SCIDs/Omenn's with an unidentified molecular basis who was transplanted from a full sib without conditioning more than 3 years ago? She is doing well clinically without serious/unusual infections. However, she has stalled in donor chimerism to only 10-14%, her lymphocytes are 96% donor (likely 100% if it were very pure without myeloid contamination). Yet, she continues with CD3/4/8 lymphopenia (CD3 is 400-1000), mostly 400-500. Most recent CD4 were 250. CD4RTE 27%, TREC is low at 280-350 (600-700/million CD3), CD19 last was 300. She is surprisingly maintaining her own IgG level 400-500. Despite the lymphopenia, PHA is good at 60-70% functionally. She responded well to non live vaccines with protective levels against Hep B, DPT and prevnar.
So the Qs are:

1.    Is this good enough at this stage since she is clinically well?

2.    Could she get live vaccines?

3.    Or, Does she need a graft boost?....
Thanks

Aly Mageed, MD, MBA
Pediatric Blood & Marrow Transplant
Helen DeVos Children's Hospital
aly.mageed at helendevoschildrens.org




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