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

[Oscar de la Calle-Martín]

Dear Kate,

I would try to look for the possible T cell chimerism  (in order to exclude
maternal origin). Another possibility is that your ADA-SCID is progressing
to an Ommen's phenotype.

Best regards,

Oscar


2013/12/18 Sullivan, Kathleen <sullivak at mail.med.upenn.edu>


> 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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-- 
--------------------------------------------------------------------------------------------------------------------------

Dr. Oscar de la Calle-Martín
Laboratoris, Planta -2, Bloc B,
Servei d'Immunologia, Hospital de Sant Pau
C/ San Quintin 89, E-08041 Barcelona, SPAIN
MOVIL SANT PAU 37386 / 682 745 510
93 553 7265 (SECRETARIA), 93 553 7386 / 93 553 7546 (DESPACHO)
odlcalle at gmail.com

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