[CIS PIDD] [cis-pidd] Dysgammaglobulinemia?

[CIS-PIDD]

PI3K pathway defect?

Ayça Aslan Kıykım, MD
Marmara University
Pediatric Allergy and Immunology
Istanbul TURKEY


12 Şub 2016 tarihinde 00:16 saatinde, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> şunları yazdı:

> What about her complement?
>  
> Alexandros Grammatikos
> North Bristol NHS Trust
>  
> From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
> Sent: Thursday, February 11, 2016 5:36 PM
> To: CIS-PIDD
> Subject: [cis-pidd] Dysgammaglobulinemia?
>  
> Colleagues,
> You may recall that last fall I posted a pregnant woman with a significant infection history and a low IgG. 
>  
> Her history includes:
> septic arthritis, N. meningitidis meningitis after vaccination, five episodes of pneumonia including an MRSA and a Klebsiella, multiple episodes of bronchitis and three episodes of adenopathy sufficient to merit biopsy - all reactive lymphoid hyperplasia. She has had some evaluation in the past that is not yet available. She has been said to be vaccine non-responsive. Post partum she has had recurrent mastitis. She mentioned that her father (a physician) has recurrent infection as well but not as troublesome as hers.
>  
> After delivering her baby (mild idiopathic CD3 lymphopenia), I extended her evaluation:
>  
> IgA=129, IgG=526, IgM=256
>  
> Serum protein electrophoresis - normal, ANA negative
>  
> Response to PPV was not robust but was adequate, 17/23. DT response adequate as well.
>  
> T cells are slightly elevated but CD 3, 4, and 8, CD 4 and 8 memory and naive cells, CD4 naive CD31+/- were normal as were CD4 and CD8 TEMRA. CD4 memory Tcm was slightly elevated and CD8 Tem was slightly low.
>  
> B cells - Total memory, class switched memory, non-switched memory and naive were all normal.
>  
> So, I have this 29 year old woman with a pretty significant infection history and not much to support significant antibody deficiency. Suggestions for the best next steps?
>  
> Richard Wasserman
> Dallas
>  
> --
> Richard L. Wasserman, MD, PhD
> Allergy Partners of North Texas
> 7777 Forest Lane, Suite B-332
> Dallas, Texas 75230
> Office (972) 566-7788
> Fax (972) 566-8837
> Cell (214) 697-7211
> ---
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