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

[Howard Lederman]

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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