[CIS PIDD] [cis-pidd] Jacobsen syndrome

[CIS-PIDD]

I am looking for some tips on followup of an infant with Jacobsen syndrome who was identified with T cell lymphopenia on newborn screening for SCID.


Her original CD4 count was around 350, with normal mitogen functions.  We did not put her on prophylaxis and have maintained a normal immunization schedule and she has been well.  Slow weight gain, but improved now with NG feeds.  We repeated her lymphocyte phenotyping this week (she is now 8 months) and her absolute CD4 count is 277.  As expected with a thymic defect her B and NK cell # are higher than normal.  She has mild thrombocytopenia (plt around 90,000).


As she nears her first birthday and would be due for her MMR and varicella vaccines, would there be any risk to her in getting these live attenuated viral vaccines?


(We have a fairly low threshold with our DiGeorge syndrome patients and will usually go ahead as long as the CD4 count is between 400-500 - but I don't know if this translates to other syndromes)


Thanks

Lisa



Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Marcus Professor of Immunology
Section, Allergy/Immunology

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