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

[Sullivan, Kathleen]

To all of you who gave me advice and guidance, thank you.

The baby had autologous cells that emerged after PEG-ADA that caused the GVHD-like picture. She did much better with some steroids.

Kate
On Dec 18, 2013, at 1:56 PM, Notarangelo, Luigi wrote:


> 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

> 

> Sent from my iPhone

> 

> 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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Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



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