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

[Notarangelo, Luigi]

Kate,

I have not seen maternal engraftment in ADA def, but I have had many cases developing Omenn-like features or signs of immune dysregulation with autologous oligoclonal cells

Gigi

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Luigi D. Notarangelo, MD
Jeffrey Modell Chair of Pediatric Immunology Research
Division of Immunology
Children's Hospital Boston
Professor of Pediatrics and Pathology
Harvard Medical School
Karp Building, Room 10217
1 Blackfan Circle
Boston, MA 02115

Tel: (617)-919-2276
Fax: (617)-730-0709



> On Dec 18, 2013, at 13:19, "Sullivan, Kathleen" <sullivak at mail.med.upenn.edu> wrote:

> 

> 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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