[PAGID] patient who lacks long-term memory antibody responses

Prof. Dr. Antonio Condino Neto condino at icb.usp.br
Fri Jul 17 10:40:26 EDT 2009


I have one 8 year boy and his 6 year old sister with persistent
respiratory infections. Both lack anti-pnemococcal antibodies.

After long term Bactrim the boy developed S Johnson Sndr and almost died.

So decided to start IVIG for both and they are very fine now.

Have no idea about the molecular defect

--
Antonio Condino-Neto, MD, PhD
Associate Professor - Department of Immunology
Institute of Biomedical Sciences - University of São Paulo
1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil
Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando "Chinen, Javier" <jxchinen at texaschildrens.org>:


> One of my Hyper IgE patients (STAT3 mutation confirmed) and another

> with a not identified genetic syndrome (extensive work up done) have

> the same phenotype. The HIES patient was on IVIG, now he is well

> with just antibiotic prophylaxis, decisions on IVIG were made based

> on family comfort with the potential risk of overwhelming infection

> and access to medical care. The second patient is OK with antibiotic

> prophylaxis (Bactrim in both cases).

> Lack of memory humoral responses is reported in HIES. It would be

> interesting to check CD27 B cells.

> Javier

> ________________________________

> From: pagid-bounces at list.clinimmsoc.org

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Howard M

> Lederman

> Sent: Thursday, July 16, 2009 1:50 PM

> To: PAGID LISTSERV

> Subject: [PAGID] patient who lacks long-term memory antibody responses

>

>

> I have a fascinating 12 yr old male patient with a history of

> recurrent infections (mostly upper and lower respiratory tract). No

> individual infection has been overwhelmingly severe, but the sum of

> all infections leads to a significant number of school absences.

>

> He has low IgG and IgM levels with normal IgA levels. He makes

> normal IgG antibody responses to a wide variety of vaccines, but

> does not seem to be able to maintain long-term immunity. (See

> attached spreadsheet. Sheet 1 tabulates the antibody and

> immunglobulin levels; sheet 2 lists the immunizations.)

>

> Is this a pattern that others have seen? I am open to any ideas

> for further evaluation. Would anyone offer this child gamma globulin?

>

> 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 jhem.jhmi.edu

>

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