[CIS-PAGID] AT patients with T-cell deficiency

[Church, Joseph]

Thank you, Howard.  

This is very valuable information.  

Do you give Ig replacement therapy to patients who do not make
antibodies to polysaccharide antigens?

JC

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Howard Lederman
Sent: Tuesday, August 02, 2011 6:00 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] AT patients with T-cell deficiency

Joe,



I have seen about 400 A-T patients over the past 12 years since we set
up our A-T Clinical Center at Hopkins.  Most of them have low T-cell
counts, frequently with CD4 counts < 400.



The only opportunistic infections that I have seen have been warts and
molluscum, sometimes very extensive and quite resistant to treatment.



I have not seen an A-T patient with PCP except in a handful (definitely
fewer than 5) of patients who were on chronic steroids for autoimmune
disease or on chemotherapy.  I am aware of one case of PCP in a severely
malnourished A-T patient with low CD4 counts.



I do not prescribe PCP prophylaxis unless the CD4 count is low and the
patient has one of those other mitigating factors.



Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology
Division of Pediatric Allergy and Immunology
Johns Hopkins Hospital - CMSC 1102
600 N. Wolfe Street
Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhem.jhmi.edu>

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________________________________
From: pagid-bounces at list.clinimmsoc.org
[pagid-bounces at list.clinimmsoc.org] on behalf of Church, Joseph
[JChurch at chla.usc.edu]
Sent: Tuesday, August 02, 2011 4:54 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] AT patients with T-cell deficiency

Colleagues:

I follow a number of Ataxia Telangietasia patients, and several have low
T-cell numbers (e.g. CD4+ ~400), normal mitogen responses, but no
antigen-specific lymphoproliferative responses.

Have any of you had AT patients who have developed opportunistic
infections such as Pneumocystis pneumonia, disseminated mycobacterial
disease, or disseminated CMV disease?

Joe Church
Children's Hospital Los Angeles

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