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

[Eleonora Gambineri]

Dear Howard,

I am following up a 8 yo boy with similar clinical presentation than  
yours. He has been suffering from recurrent/chronic sinusitis since a  
couple of year (before he was healthy). Recently he also suffered from  
pneumonia.

Hereby his labs:

CBC and differential: normal
LFTs: normal
Creatinine: normal

Vaccinal antibodies:
Anti-tet: neg  (last booster 2 yrs ago)
Anti-measles: neg
Anti-dift: neg
Anti-HIV: neg
Anti-pneumococcus: ongoing

CMV Ab: IgG POS;               PCR CMV (peripheral blood): neg
EBV Ab: neg;                         PCR EBV (peripheral blood): neg


IgG 1240 mg/dl
IgA 166
IgM 179

IgE 34 kU/L

IgG1   1070 mg/dl
IgG2    140
IgG3    88
IgG4    2

Anti-nuclear Ab: neg

His T cell phenotype showed low CD4 count (400-500/ul) mainly CD45 RO  
memory CD4 T cells (65%). The B cell phenotype showed a normal  
differentiation of memory B cells except for increased transitional B  
cells (9,8%).

He is at the moment in prophylaxis with Azithromycin: 350 mg 3 times/ 
week. No infections so far.

Before considering IVIG I will see how he will do clinically. If he  
need to stay on antibiotics to be free from infections I will probably  
consider immunoglobulin replacement.

Since the thymic output seems normal (most of CD4+ CD45RA+ cells are  
CD31+) I was also considering other condition with helper T cell- 
dependent antibody defects i.e. XLP ? Although I am also open to any  
kind of suggestions!!

Best wishes,
Ele

*******************************************************************
Dott.ssa Eleonora Gambineri
Ricercatore Universitario
Universita' degli Studi di Firenze
Dipartimento di Pediatria-Ospedale Pediatrico "Anna Meyer"
Viale Gaetano Pieraccini, 24
50139 FIRENZE
Tel 055 5662405 (ufficio)
        055 5662464 (lab)
Fax 055 4221012
e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it

Eleonora Gambineri, MD
Researcher/Assistant Professor
University of Florence
Department of Pediatrics-"Anna Meyer" Children's Hospital
Viale Gaetano Pieraccini, 24
50139 FIRENZE
ITALY
Tel +39 055 5662405 (office)
        +39 055 5662464 (lab)
Fax +39 055 4221012
e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
********************************************************************





On 19/lug/09, at 03:08, Berger, Melvin wrote:


> 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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