[PAGID] an interesting case from Turkey

[John Ziegler]

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with normal IgA and IgM it would be unusual to have low IgG. Can you get it
done in a more sensitive assay?

  _____  

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Yesim Yilmaz
Sent: Monday, 22 February 2010 2:08 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] an interesting case from Turkey


thanks for noticing:

Igs were checked 3 times, IgG values were less than 300 mg/dl in all of them
(this is all I have)

the lymph. subpopulation: CD3 36%, CD8: 9 %, CD4: 30%, CD20: 44%, CD56/16:
14% (I will ask the absolute values)

thanks again,

Yeshim




2010/2/21 John Ziegler <j.ziegler at unsw.edu.au>


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The Igs could be normal (if IgG close to 300 mg/dL). DO you have another
quantitation. Could you confirm the units are mg/dL.
 
Did you mean CD3 36% , CD4 9% CD8: 30 % (or CD8 9% CD4 30%) or something
else.
 


  _____  


From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Yesim Yilmaz

Sent: Saturday, 20 February 2010 1:55 AM 

To: pagid at list.clinimmsoc.org
Subject: [PAGID] an interesting case from Turkey


Dear all,
 
I am posting this interesting case for my former mentor in Turkey: 
 
the patient is a 10 month-old boy
parents are fist cousins 
he was born at 36 wks EGA, stayed in NICU at a local hospital for unknown
reasons, received his vaccinations including BCG, no complications.
he started to have pneumonia, diarrhea, mucositis at 6 months of age,
cultures were negative.
his lenght & weight are below 3rd percentile
other positive physical exam findings: ABSENT THUMB, FLAT FACE
 
he frequently develops neutropenia during infections, responds to G-CSF very
well
never had thrombocytopenia or anemia
MRI of the pancreas is normal, he doesn't have steatorrhea
no bone abnormalities on X-ray
  
IgG <300 mg , IgM:63 IgA:110 IgE 7 İU  
CD3 36% , CD49: % CD4: 30 % CD20 44 % CD 16/56 14 %
 
normal albumin, no significant loss after IVIG.
 
 
my mentor found only one case report similar to this case:
 
Short stature, absent thumbs, flat facies, anosmia
<http://www.ncbi.nlm.nih.gov/pubmed/728551?itool=EntrezSystem2.PEntrez.Pubme
d.Pubmed_ResultsPanel.Pubmed_RVDocSum&ordinalpos=24> and combined immune
deficiency (CID). 

Shokeir MH.

Birth Defects Orig Artic Ser. 1978;14(6A):103-16. 

thank you very much for any comments and input.

Yeshim 

 

Yesim Yilmaz Demirdag, MD

Assistant Professor

Section of Allergy and Immunology

Department of Pediatrics

West Virginia University School of Medicine

Morgantown, WV

phone: (304) 293 1201   

e-mail: ydemirdag at hsc.wvu.edu



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