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

[grumach at usp.br]

We have been following a factor H deficient patient with pneumococcal  
infections. After the first pneumococcal vaccine he had no antibody  
response. We prescribed antibiotics. Conjugated vaccine and non  
conjugated vaccine were repeated with improvement. So, I agree that  
CH50, C3 and C4 should be evaluated.

Anete S Grumach, MD, PhD
Outpatient group of Primary Immunodeficiencies
Dept of Dermatology, Univ of Sâo Paulo
and Dept of Pneumology, Faculty of Medicine ABC


Citando "Berger, Melvin" <Melvin.Berger at UHhospitals.org>:


> C4 deficiency causes the inability to make memory responses to   

> T-dependent antigens. Each time they are immunized they make a   

> repeated primary response, but even after several immunizations   

> there is still only a little IgG, and the amount doesn't increase.   

> This was well documented by Hans Ochs in a New england journal paper  

>  about 20 yeatrs ago (or more) with phage. Evebtually, the patients'  

>  IgG drops- this also occurs in C3 deficiency.  I would definitely   

> check C3 and C4 if you don't have those results.

>

> On the IGIV issue, he does seem to maintain Abs against quite a few   

> antigens. I might try prophylactic antibiotics before an empirical   

> trial of IgG supplementation if the former is not enough. If there   

> is an outbreak of measles, he should probably be given a dose of IgG.

>

> Melvin Berger, M.D., Ph.D.

> Adjunct Professor of Pediatrics and Pathology

> Case Western Reserve University

> phone 216 844 3237

>

> Director, Jeffrey Modell Center for Primary Immune Deficiencies

> Division of Allergy-Immunology

> Rainbow, Babies and Children's Hospital

> University Hospitals of Cleveland

> RB&C Rm 504, MS 6008B

> 11100 Euclid Ave.

> Cleveland, OH 44106

>

> ________________________________

>

> From: pagid-bounces at list.clinimmsoc.org on behalf of Howard M Lederman

> Sent: Thu 7/16/2009 2:49 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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