[CIS-PAGID] ? Combined Immune Deficiency

[Church, Joseph]

Colleagues:

 

I follow a 5yo boy who presented with recurrent pneumonias and failure
to thrive since 8 months of age.  At 1 year he developed severe
varicella after immunization with VZV.

 

Normal tests include:

*        T and B and NK cell numbers

*        Mitogen lymphoproliferative (LPA) responses

*        Tetanus, Candida LPA

*        IgG and IgA

*        Tetanus and Hib antibody responses

*        Varicella antibody responses

*        NK cell cytotoxicity (Cincinnati Children's)

*        HLA/MHC I and II expression

 

Abnormal tests include:

*	Elevated percentage of B-cells (~30%)

*	Borderline LPA to Varicella virus
*	Borderline IgM levels
*	Poor antibody responses to Pneumovax
*	ABSENT CTL function x2 (Cincinnati Children's)

 

Currently, he is doing well on IVIG.

 

Does this patient fit with any known syndromes?

 

Thanks for your help.

 

Joe Church

Children's Hospital Los Angeles



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