[PAGID] (no subject)

[Richard Hong]

John

What kind of anemia is it? A primary red cell aplasia is often a sign of T 
cell abnormality.
If she is losing a lot of goodies via the gut she should have 
hypoalbuminemia; transferrin is really the best protein measure for GI loss, 
however.
I'm impressed that she has reasonable amounts of IgA and IgM in the face of 
the low IgG. That is a strange pattern. Still she seems to have NO response 
to any of the antigens. Why? Did you do mitogenic and mixed leukocyte 
responses to get a view of "functional" T cell capabilities? These responses 
suggest that she has the immunocytes but they are not in an environment 
supporting their function.
Don't keep up with all of the newer varieties of enzyme defects, but is 
there something new in the area of the ADA, PNP type of metabolic causes?

Dick Hong
University of Vermont
----- Original Message ----- 
From: "Sullivan, John (Office of Research)" <John.Sullivan at umassmed.edu>
To: "PAGID Consult (E-mail)" <pagid at clinimmsoc.org>
Sent: Thursday, August 03, 2006 2:37 PM
Subject: [PAGID] (no subject)


> Colleagues, I would appreciate your thoughts about the following patient; 
> a 16 month old female who presented on July 16th, with a severe anemia[hgb 
> 6], FTT[ wt<3%, HT at 75%] neutropenia[ <5%] and diarrhea; IgG 165, IgA 
> 19, IgM 54, IgE 91; normal lymphocyte count with normal T cells and normal 
> CD4/CD8, normal B cell numbers; patient has had all appropriate 
> immunizations and has absent antibodies to the following; 
> tetanus/diphtheria/strep pneumo/hep B/H Flu/ and Varicella; patient 
> transfused and has stable hematocrit and will receive IVIG tomorrow[ just 
> received results of immune workup]; thanks, John.
>
>
> John L Sullivan MD
> Professor of Pediatrics
> University of Massachusetts Medical School
> 55 Lake Avenue North
> Worcester MA 01655
> Phone 508-856-1572
> Fax 508-856-5004
> email john.sullivan at umassmed.edu
>