[CIS PIDD] [cis-pidd] Live Vaccines for AT babies diagnosed by SCID screen?

[CIS-PIDD]

In 1992 (PMID 1517916) we described one of last cases of paralytic polio in Canada, developing in an unimmunized 10 month old after exposure to his cousin who had recently received live poliio vaccine.  Ig levels and antibody response to the infecting vaccine strain were documented in our patient; no lymphocyte studies were done but his lymphocyte count was normal.  Three years later he returned with classic signs of AT, a low lymphocyte count and low T and B cells, but normal Ig levels and positive antibody responses to vaccines he'd received including DT and MMR, and normal mitogen-induced lymphoproliferative responses.  He was subsequently genetically confirmed to have AT and years later succumbed to lymphoproliferative disease.  So an area "where angels fear to treat.."    anne

Anne Junker, MD
Associate Professor, Pediatrics
BC Children's Hospital and University of British Columbia
Vancouver, BC, Canada
ajunker at cw.bc.ca
________________________________________
From: cis-pidd at lyris.dundee.net [cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, May 10, 2018 10:41 AM
To: CIS-PIDD
Subject: [cis-pidd] Live Vaccines for AT babies diagnosed by SCID screen?

Hello all,
Now that we are all diagnosing AT babies very early, is there a consensus on live vaccines?
Are people just doing it case by case based on CD4 and CD8 counts as well as proliferation?
Would people be willing to share what they do at their institution?

We just diagnosed a baby with AT who was picked up on NBS.  He is due for his 2 month vaccines.
CD4 count around 800, CD8 count count only 250, B cells very low around 80 cells/ul, normal NK cells,
Very decent proliferation with PHA and Pokeweed.
I want to say ok, but now with this concern about rubella positive granulomas in patients with AT linked with MMR, I am wondering if I should be more cautious.

Thank you for your thoughts,

Hey

Hey Jin Chong MD PhD
Division Director of Pediatric Allergy & Immunology
Assistant Professor of Pediatrics
Children's Hospital of Pittsburgh of UPMC
One Children's Hospital Drive
4401 Penn Avenue
Pittsburgh, PA 15224
tel 412-692-7885
fax 412-692-8499





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