[PAGID] PAGID posting

[dmvascon at usp.br]

Dear Dr. Wickner

I think that your patient has a very severe form of immunodeficiency  
to be classified as a CVID. Despite the low levels of IgG2 and IgA,  
his cellular dysfunction is apparently very precocious for CVID. In  
our experience CVID patients usually present cellular deficits  
(initially T cells and afterwards NK cells) later in life, generally  
after 50 years of age, at least 25 years of clinical disease and in  
the patients that have high CD8 T cell counts and splenomegaly.  
Apparently his immune function is deteriorating very quickly (in one  
year his lymphoproliferation to mitogens is falling) with clinical  
evidence of cellular immunodeficiency - shingles and candida  
esophagitis; despite the fact that is important to correlate these  
findings - clinical and laboratorial - with the possible use of  
corticosteroids for the control of asthma.
Does he have a abnormal facies or any other characteristic phenotype,  
to think about ICL or other DNA repair defect (AT, Nijmegen) that  
usually present a progressive disease?
As a matter of fact I would think initially in a mild form of CID with  
progressive features, such as ADA deficiency, or another variant of a  
leaky SCID with NK cell deficiency. Moreover the very low counts of  
CD4/CD45RA cells for age are suggestive of a low thymic output  
favoring a CID diagnosis.
Please keep us posted on the evolution and evaluation of the patient.

Kind regards,

Dewton de Moraes-Vasconcelos
University of São Paulo School of Medicine


Citando Paige wickner <paigewickner at gmail.com>:


> Hi - I wanted to post the attached case to the list. Thank you! I havent

> submitted a case before- so is this how it works?

> Thank you!

> Paige Wickner

> Allergy/Immunology Fellow

> Yale New Haven Hospital

>


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